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- Product number
- 10782-2-AP - Provider product page
- Provider
- Proteintech Group
- Proper citation
- Proteintech Cat#10782-2-AP, RRID:AB_615042
- Product name
- TDP-43 antibody
- Antibody type
- Polyclonal
- Description
- KD/KO validated TDP-43 antibody (Cat. #10782-2-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat, zebrafish, kak and has been validated for the following applications: FC, IF, IHC, WB, ELISA.
- Reactivity
- Human, Mouse, Rat, Zebrafish
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 20ul, 150ul
Submitted references Pathological Markers of Alzheimer's Disease and Related Dementia in the Rhesus Macaque Amygdala.
Evaluation of cyanotoxin L-BMAA effect on α-synuclein and TDP43 proteinopathy.
Biochemical, Biomarker, and Behavioral Characterization of the Grn(R493X) Mouse Model of Frontotemporal Dementia.
Pathologic TDP-43 downregulates myelin gene expression in the monkey brain.
Involvement of CB(1) and CB(2) receptors in neuroprotective effects of cannabinoids in experimental TDP-43 related frontotemporal dementia using male mice.
A TBK1 variant causes autophagolysosomal and motoneuron pathology without neuroinflammation in mice.
The new missense G376V-TDP-43 variant induces late-onset distal myopathy but not amyotrophic lateral sclerosis.
Serum GFAP levels correlate with astrocyte reactivity, post-mortem brain atrophy and neurofibrillary tangles.
RNA-binding properties orchestrate TDP-43 homeostasis through condensate formation in vivo.
Time-resolved profiling of RNA binding proteins throughout the mRNA life cycle.
CAG repeat mosaicism is gene specific in spinocerebellar ataxias.
DBT is a metabolic switch for maintenance of proteostasis under proteasomal impairment.
Oxidative cyclization reagents reveal tryptophan cation-π interactions.
Colocalization of TDP-43 and stress granules at the early stage of TDP-43 aggregation in amyotrophic lateral sclerosis.
Casein kinase 1 inhibitor avoids TDP-43 pathology propagation in a patient-derived cellular model of amyotrophic lateral sclerosis.
Large-scale RNA-seq mining reveals ciclopirox triggers TDP-43 cryptic exons.
The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD.
Endogenous TDP-43 mislocalization in a novel knock-in mouse model reveals DNA repair impairment, inflammation, and neuronal senescence.
Reduced STMN2 and pathogenic TDP-43, two hallmarks of ALS, synergize to accelerate motor decline in mice.
Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons.
Regulating PCCA gene expression by modulation of pseudoexon splicing patterns to rescue enzyme activity in propionic acidemia.
Computational prediction and experimental validation identify functionally conserved lncRNAs from zebrafish to human.
Loss of TDP-43 mediates severe neurotoxicity by suppressing PJA1 gene transcription in the monkey brain.
Cryptic splicing of stathmin-2 and UNC13A mRNAs is a pathological hallmark of TDP-43-associated Alzheimer's disease.
Cross-seeding by prion protein inactivates TDP-43.
TDP43/HDAC6/Prdx1 signaling pathway participated in the cognitive impairment of obstructive sleep apnea via regulating inflammation and oxidative stress.
TDP-43 nuclear loss in FTD/ALS causes widespread alternative polyadenylation changes.
TDP-43 impairs sleep in Drosophila through Ataxin-2-dependent metabolic disturbance.
TDP-43 pathology links innate and adaptive immunity in amyotrophic lateral sclerosis.
IκB kinase phosphorylates cytoplasmic TDP-43 and promotes its proteasome degradation.
Mouse oocytes sequester aggregated proteins in degradative super-organelles.
hnRNPA2B1 represses the disassembly of arsenite-induced stress granules and is essential for male fertility.
Age-Related Pathology in Corticobasal Degeneration.
Restoring functional TDP-43 oligomers in ALS and laminopathic cellular models through baicalein-induced reconfiguration of TDP-43 aggregates.
Biochemical, biomarker, and behavioral characterization of the Grn(R493X) mouse model of frontotemporal dementia.
Regulation of stress granule formation in human oligodendrocytes.
Reduced progranulin increases tau and α-synuclein inclusions and alters mouse tauopathy phenotypes via glucocerebrosidase.
Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia.
A Panel of miRNA Biomarkers Common to Serum and Brain-Derived Extracellular Vesicles Identified in Mouse Model of Amyotrophic Lateral Sclerosis.
Retrospective neuropathological diagnosis of TDP-43 proteinopathies: Factors affecting immunoreactivity of phosphorylated TDP-43 in fixed post-mortem brain tissue.
Exploring Options for Proximity-Dependent Biotinylation Experiments: Comparative Analysis of Labeling Enzymes and Affinity Purification Resins.
KCNJ2 inhibition mitigates mechanical injury in a human brain organoid model of traumatic brain injury.
Seeding competent TDP-43 persists in human patient and mouse muscle.
Design principles to tailor Hsp104 therapeutics.
Depletion of Mettl3 in cholinergic neurons causes adult-onset neuromuscular degeneration.
Reversal of C9orf72 mutation-induced transcriptional dysregulation and pathology in cultured human neurons by allele-specific excision.
Anterior insula is more vulnerable than posterior insula to TDP-43 pathology in common dementias and ALS.
TDP-43 deficiency in suprachiasmatic nucleus perturbs rhythmicity of neuroactivity in prefrontal cortex.
Targeting 14-3-3θ-mediated TDP-43 pathology in amyotrophic lateral sclerosis and frontotemporal dementia mice.
VCP-related myopathy: a case series and a review of literature.
Sedimentation Assays to Assess the Impact of Posttranslational Modifications on Phase Separation of RNA-Binding Proteins In Vitro and In Cells.
Protocol for the Systematic Fixation, Circuit-Based Sampling, and Qualitative and Quantitative Neuropathological Analysis of Human Brain Tissue.
Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis.
Loss of TDP-43 function contributes to genomic instability in amyotrophic lateral sclerosis.
TTBK1 and CK1 inhibitors restore TDP-43 pathology and avoid disease propagation in lymphoblast from Alzheimer's disease patients.
Codon-optimized TDP-43 mediates neurodegeneration in a Drosophila model of ALS/FTLD.
Differential effects on TDP-43, piezo-2, tight-junction proteins in various brain regions following repetitive low-intensity blast overpressure.
The identification of high-performing antibodies for TDP-43 for use in Western Blot, immunoprecipitation and immunofluorescence.
Increased oligomeric TDP-43 in the plasma of Korean frontotemporal dementia patients with semantic dementia.
A panel of TDP-43-regulated splicing events verifies loss of TDP-43 function in amyotrophic lateral sclerosis brain tissue.
Loss of TDP-43 oligomerization or RNA binding elicits distinct aggregation patterns.
Su(var)3-9 mediates age-dependent increase in H3K9 methylation on TDP-43 promoter triggering neurodegeneration.
iPSC motor neurons, but not other derived cell types, capture gene expression changes in postmortem sporadic ALS motor neurons.
Causal ALS genes impact the MHC class II antigen presentation pathway.
Cytotoxic immune cells do not affect TDP-43 and p62 sarcoplasmic aggregation but influence TDP-43 localisation.
TDP43 pathology in chronic traumatic encephalopathy retinas.
Tuning Hsp104 specificity to selectively detoxify α-synuclein.
Selective Noradrenaline Depletion in the Neocortex and Hippocampus Induces Working Memory Deficits and Regional Occurrence of Pathological Proteins.
Rare exonic variant affects GRN splicing and contributes to frontotemporal lobar degeneration.
Human herpesvirus 8 ORF57 protein is able to reduce TDP-43 pathology: network analysis identifies interacting pathways.
circPSD3 is a promising inhibitor of uPA system to inhibit vascular invasion and metastasis in hepatocellular carcinoma.
Amyloidogenic propensity of self-assembling peptides and their adjuvant potential for use as DNA vaccines.
A review on neurodegeneration in the fast-ageing killifish, the first animal model to study the natural occurrence of neuronal cell loss.
m(1)A in CAG repeat RNA binds to TDP-43 and induces neurodegeneration.
Different Chronic Stress Paradigms Converge on Endogenous TDP43 Cleavage and Aggregation.
Dysregulation of the progranulin-driven autophagy-lysosomal pathway mediates secretion of the nuclear protein TDP-43.
Chronic exposure to l-BMAA cyanotoxin induces cytoplasmic TDP-43 accumulation and glial activation, reproducing an amyotrophic lateral sclerosis-like phenotype in mice.
G(2)C(4) targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons.
Time-resolved proteomic profiling reveals compositional and functional transitions across the stress granule life cycle.
Targeted perturbation of signaling-driven condensates.
Creation of de novo cryptic splicing for ALS/FTD precision medicine.
TDP-43 knockdown in mouse model of ALS leads to dsRNA deposition, gliosis, and neurodegeneration in the spinal cord.
Preclinical investigation in FAAH inhibition as a neuroprotective therapy for frontotemporal dementia using TDP-43 transgenic male mice.
A New Mouse Model of Giant Axonal Neuropathy with Overt Phenotypes and Neurodegeneration Driven by Neurofilament Disorganization.
Looking for answers far away from the soma-the (un)known axonal functions of TDP-43, and their contribution to early NMJ disruption in ALS.
Tandem detergent-extraction and immunoprecipitation of proteinopathy: Scalable enrichment of ALS-associated TDP-43 aggregates.
Elevated nuclear TDP-43 induces constitutive exon skipping.
SULT1A1-dependent sulfonation of alkylators is a lineage-dependent vulnerability of liver cancers.
MicroRNA-183-5p regulates TAR DNA-binding protein 43 neurotoxicity via SQSTM1/p62 in amyotrophic lateral sclerosis.
Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging.
Deficiency of Perry syndrome-associated p150(Glued) in midbrain dopaminergic neurons leads to progressive neurodegeneration and endoplasmic reticulum abnormalities.
Loss of C9orf72 perturbs the Ran-GTPase gradient and nucleocytoplasmic transport, generating compositionally diverse Importin β-1 granules.
Development of FRET and Stress Granule Dual-Based System to Screen for Viral 3C Protease Inhibitors.
Aggregation-prone TDP-43 sequesters and drives pathological transitions of free nuclear TDP-43.
ILF3 prion-like domain regulates gene expression and fear memory under chronic stress.
Sustained therapeutic benefits by transient reduction of TDP-43 using ENA-modified antisense oligonucleotides in ALS/FTD mice.
Ballooned neurons in semi-recent severe traumatic brain injury.
Macroglossia in rapidly progressive inclusion body myositis.
Viral vector gene delivery of the novel chaperone protein SRCP1 to modify insoluble protein in in vitro and in vivo models of ALS.
Early activation of cellular stress and death pathways caused by cytoplasmic TDP-43 in the rNLS8 mouse model of ALS and FTD.
Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage.
CircTmeff1 Promotes Muscle Atrophy by Interacting with TDP-43 and Encoding A Novel TMEFF1-339aa Protein.
Suppression of TDP-43 aggregation by artificial peptide binder targeting to its low complexity domain.
Molecular and Phenotypic Changes in FLExDUX4 Mice.
The influence of age and sex on the absolute cell numbers of the human brain cerebral cortex.
Disruption of ER ion homeostasis maintained by an ER anion channel CLCC1 contributes to ALS-like pathologies.
Nuclear Expression of TDP-43 Is Linked with Morphology and Ubiquitylation of Cytoplasmic Aggregates in Amyotrophic Lateral Sclerosis.
Aberrant dynein function promotes TDP-43 aggregation and upregulation of p62 in male mice harboring transgenic human TDP-43.
Specific vulnerability of iPSC-derived motor neurons with TDP-43 gene mutation to oxidative stress.
Neuroimmune characterization of optineurin insufficiency mouse model during ageing.
Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo.
Atypical TDP-43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP.
Neuropathology of central nervous system involvement in TTR amyloidosis.
Tuberous sclerosis complex is associated with a novel human tauopathy.
Study of Ubiquitin Pathway Genes in a French Population with Amyotrophic Lateral Sclerosis: Focus on HECW1 Encoding the E3 Ligase NEDL1.
Stress granule assembly in vivo is deficient in the CNS of mutant TDP-43 ALS mice.
A fluid biomarker reveals loss of TDP-43 splicing repression in pre-symptomatic ALS.
Rapid nuclear deadenylation of mammalian messenger RNA.
RNA methylation influences TDP43 binding and disease pathogenesis in models of amyotrophic lateral sclerosis and frontotemporal dementia.
Amyotrophic lateral sclerosis with TDP-43 abnormalities exhibiting globular glial tau inclusions in frontotemporal lobes and pallido-nigral system.
Loss of TDP-43 function underlies hippocampal and cortical synaptic deficits in TDP-43 proteinopathies.
Quantitative proteomics of tau and Aβ in detergent fractions from Alzheimer's disease brains.
Distinctive chaperonopathy in skeletal muscle associated with the dominant variant in DNAJB4.
Progranulin Deficiency Induces Mitochondrial Dysfunction in Frontotemporal Lobar Degeneration with TDP-43 Inclusions.
Endogenous retroviruses and TDP-43 proteinopathy form a sustaining feedback driving intercellular spread of Drosophila neurodegeneration.
SYF2 suppression mitigates neurodegeneration in models of diverse forms of ALS.
Functional Characterization of a Familial ALS-Associated Missense TBK1 (p-Arg573Gly) Mutation in Patient-Derived Lymphoblasts.
MATR3 P154S knock-in mice do not exhibit motor, muscle or neuropathologic features of ALS.
PIKFYVE inhibition mitigates disease in models of diverse forms of ALS.
Ataxin-2 polyglutamine expansions aberrantly sequester TDP-43, drive ribonucleoprotein condensate transport dysfunction and suppress local translation.
Exosomal TAR DNA binding protein 43 profile in canine model of amyotrophic lateral sclerosis: a preliminary study in developing blood-based biomarker for neurodegenerative diseases.
An Autopsy Series of Seven Cases of VPS13A Disease (Chorea-Acanthocytosis).
GM1 ganglioside exerts protective effects against glutamate-excitotoxicity via its oligosaccharide in wild-type and amyotrophic lateral sclerosis motor neurons.
Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons.
Loss of TDP-43 splicing repression occurs early in the aging population and is associated with Alzheimer's disease neuropathologic changes and cognitive decline.
Highly variable molecular signatures of TDP-43 loss of function are associated with nuclear pore complex injury in a population study of sporadic ALS patient iPSNs.
Repeated mild traumatic brain injury triggers pathology in asymptomatic C9ORF72 transgenic mice.
TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP.
Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP-43 overexpression mouse model of amyotrophic lateral sclerosis.
Psychotic symptoms in frontotemporal dementia with TDP-43 tend to be associated with type B pathology.
Comparison of TRIBE and STAMP for identifying targets of RNA binding proteins in human and Drosophila cells.
N protein of PEDV plays chess game with host proteins by selective autophagy.
Chaperone-mediated autophagy in neuronal dendrites utilizes activity-dependent lysosomal exocytosis for protein disposal.
Astrocytic response mediated by the CLU risk allele inhibits OPC proliferation and myelination in a human iPSC model.
Traumatic brain injury induces TDP-43 mislocalization and neurodegenerative effects in tissue distal to the primary injury site in a non-transgenic mouse.
Reduction of nemo-like kinase increases lysosome biogenesis and ameliorates TDP-43-related neurodegeneration.
Hyperphosphorylated Tau in Mesial Temporal Lobe Epilepsy: a Neuropathological and Cognitive Study.
TDP-43 pathology and functional deficits in wild-type and ALS/FTD mutant cyclin F mouse models.
Severe cerebrovascular pathology of the first supercentenarian to be autopsied in the world.
Autophagic vacuolar myopathy involving the phenotype of spinocerebellar ataxia type 3.
Astrocytic TDP-43 dysregulation impairs memory by modulating antiviral pathways and interferon-inducible chemokines.
Modulation of tau tubulin kinases (TTBK1 and TTBK2) impacts ciliogenesis.
Neuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding.
Argyrophilic grain disease is common in older adults and may be a risk factor for suicide: a study of Japanese forensic autopsy cases.
Entorhinal Subfield Vulnerability to Neurofibrillary Tangles in Aging and the Preclinical Stage of Alzheimer's Disease.
Allele-specific silencing as therapy for familial amyotrophic lateral sclerosis caused by the p.G376D TARDBP mutation.
TDP-43 Oligomerization and Phase Separation Properties Are Necessary for Autoregulation.
DNA damage stress-induced translocation of mutant FUS proteins into cytosolic granules and screening for translocation inhibitors.
ATP-binding cassette transporter expression is widely dysregulated in frontotemporal dementia with TDP-43 inclusions.
C9orf72 regulates the unfolded protein response and stress granule formation by interacting with eIF2α.
TDP-43 Pathology Exacerbates Cognitive Decline in Primary Age-Related Tauopathy.
Hyperphosphorylation-Mimetic TDP-43 Drives Amyloid Formation and Possesses Neuronal Toxicity at the Oligomeric Stage.
Intracellular energy controls dynamics of stress-induced ribonucleoprotein granules.
Importance of the Q/N-rich segment for protein stability of endogenous mouse TDP-43.
Characterizing Lewy Pathology in 231 Essential Tremor Brains From the Essential Tremor Centralized Brain Repository.
Stress induced TDP-43 mobility loss independent of stress granules.
Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins.
Integrated stress response is involved in the 24(S)-hydroxycholesterol-induced unconventional cell death mechanism.
Genome-wide CRISPR screen reveals v-ATPase as a drug target to lower levels of ALS protein ataxin-2.
Impaired disassembly of the axon initial segment restricts mitochondrial entry into damaged axons.
Pathological 25 kDa C-Terminal Fragments of TDP-43 Are Present in Lymphoblastoid Cell Lines and Extracellular Vesicles from Patients Affected by Frontotemporal Lobar Degeneration and Neuronal Ceroidolipofuscinosis Carrying a GRN Mutation.
Robust Generation of Ready-to-Use Cryopreserved Motor Neurons from Human Pluripotent Stem Cells for Disease Modeling.
Understanding In Vitro Pathways to Drug Discovery for TDP-43 Proteinopathies.
Ubiquitin-positive astrogliopathy clinically mimicking Parkinson's disease.
Perception of Fragmented Letters by Patients With Pathologically Confirmed Dementia With Lewy Bodies or Alzheimer Disease.
Pathogenic Mutation of TDP-43 Impairs RNA Processing in a Cell Type-Specific Manner: Implications for the Pathogenesis of ALS/FTLD.
Insulin-like growth factor 5 associates with human Aß plaques and promotes cognitive impairment.
TDP43 promotes stemness of breast cancer stem cells through CD44 variant splicing isoforms.
TARDBP Inhibits Porcine Epidemic Diarrhea Virus Replication through Degrading Viral Nucleocapsid Protein and Activating Type I Interferon Signaling.
Nuclear RNA transcript levels modulate nucleocytoplasmic distribution of ALS/FTD-associated protein FUS.
Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology.
Nuclear pore destruction and loss of nuclear TDP-43 in FUS mutation-related amyotrophic lateral sclerosis motor neurons.
TDP-43 regulates cholesterol biosynthesis by inhibiting sterol regulatory element-binding protein 2.
TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A.
TDP-43 represses cryptic exon inclusion in the FTD-ALS gene UNC13A.
Sirtuin-1 sensitive lysine-136 acetylation drives phase separation and pathological aggregation of TDP-43.
RTL8 promotes nuclear localization of UBQLN2 to subnuclear compartments associated with protein quality control.
Accumulation of Nonfibrillar TDP-43 in the Rough Endoplasmic Reticulum Is the Early-Stage Pathology in Amyotrophic Lateral Sclerosis.
Therapeutic modulation of GSTO activity rescues FUS-associated neurotoxicity via deglutathionylation in ALS disease models.
An open source toolkit for repurposing Illumina sequencing systems as versatile fluidics and imaging platforms.
ADARs act as potent regulators of circular transcriptome in cancer.
Parallel Appearance of Polyglutamine and Transactivation-Responsive DNA-Binding Protein 43 and Their Complementary Subcellular Localization in Brains of Patients With Spinocerebellar Ataxia Type 2.
Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels.
NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility.
Sequestration of TDP-43(216-414) Aggregates by Cytoplasmic Expression of the proSAAS Chaperone.
Prolyl Isomerase Pin1 Expression in the Spinal Motor Neurons of Patients With Sporadic Amyotrophic Lateral Sclerosis.
A quantitative biology approach correlates neuronal toxicity with the largest inclusions of TDP-43.
Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export.
Hyperosmotic-stress-induced liquid-liquid phase separation of ALS-related proteins in the nucleus.
TREM2 interacts with TDP-43 and mediates microglial neuroprotection against TDP-43-related neurodegeneration.
Chemically induced senescence in human stem cell-derived neurons promotes phenotypic presentation of neurodegeneration.
miR-23a suppression accelerates functional decline in the rNLS8 mouse model of TDP-43 proteinopathy.
Analysis of the Biomarkers for Neurodegenerative Diseases in Aged Progranulin Deficient Mice.
Developmental deficits and staging of dynamics of age associated Alzheimer's disease neurodegeneration and neuronal loss in subjects with Down syndrome.
CHMP2B regulates TDP-43 phosphorylation and cytotoxicity independent of autophagy via CK1.
Structure-Based Analysis of Protein Cluster Size for Super-Resolution Microscopy in the Nervous System.
Feeder-supported in vitro exercise model using human satellite cells from patients with sporadic inclusion body myositis.
Loss of TDP-43 function and rimmed vacuoles persist after T cell depletion in a xenograft model of sporadic inclusion body myositis.
Sublethal enteroviral infection exacerbates disease progression in an ALS mouse model.
Effects of intracellular calcium accumulation on proteins encoded by the major genes underlying amyotrophic lateral sclerosis.
Vulnerable exons, like ACADM exon 5, are highly dependent on maintaining a correct balance between splicing enhancers and silencers.
TDP-43 Pathology and Prionic Behavior in Human Cellular Models of Alzheimer's Disease Patients.
A postzygotic de novo NCDN mutation identified in a sporadic FTLD patient results in neurochondrin haploinsufficiency and altered FUS granule dynamics.
Association of Neurofibrillary Tangle Distribution With Age at Onset-Related Clinical Heterogeneity in Alzheimer Disease: An Autopsy Study.
Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling.
TDP-43 condensates and lipid droplets regulate the reactivity of microglia and regeneration after traumatic brain injury.
Diverse limbic comorbidities cause limbic and temporal atrophy in lewy body disease.
Therapeutic Effect of Rapamycin on TDP-43-Related Pathogenesis in Ischemic Stroke.
An autopsy case of amyotrophic lateral sclerosis with striatonigral and pallidoluysian degeneration and cat's-eye-shaped neuronal nuclear inclusions.
Unraveling the toxic effects mediated by the neurodegenerative disease-associated S375G mutation of TDP-43 and its S375E phosphomimetic variant.
Aberrant neural activity in prefrontal pyramidal neurons lacking TDP-43 precedes neuron loss.
ALS-linked KIF5A ΔExon27 mutant causes neuronal toxicity through gain-of-function.
A motor neuron disease mouse model reveals a non-canonical profile of senescence biomarkers.
Testing of the therapeutic efficacy and safety of AMPA receptor RNA aptamers in an ALS mouse model.
Long-standing multiple system atrophy-Parkinsonism with limbic and FTLD-type α-synuclein pathology.
Plasma biomarkers for Alzheimer's Disease in relation to neuropathology and cognitive change.
Heterozygous frameshift variants in HNRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy.
Disease-linked TDP-43 hyperphosphorylation suppresses TDP-43 condensation and aggregation.
Homotypic fibrillization of TMEM106B across diverse neurodegenerative diseases.
Physiological tissue-specific and age-related reduction of mouse TDP-43 levels is regulated by epigenetic modifications.
Polyadenylated RNA and RNA-Binding Proteins Exhibit Unique Response to Hyperosmotic Stress.
Neuronal VPS35 deletion induces spinal cord motor neuron degeneration and early post-natal lethality.
Behavioral Deficits in Mice with Postnatal Disruption of Ndel1 in Forebrain Excitatory Neurons: Implications for Epilepsy and Neuropsychiatric Disorders.
hnRNP A1B, a Splice Variant of HNRNPA1, Is Spatially and Temporally Regulated.
Repeated Low-Level Blast Acutely Alters Brain Cytokines, Neurovascular Proteins, Mechanotransduction, and Neurodegenerative Markers in a Rat Model.
Targeted Quantification of Detergent-Insoluble RNA-Binding Proteins in Human Brain Reveals Stage and Disease Specific Co-aggregation in Alzheimer's Disease.
TDP-43 proteinopathy impairs mRNP granule mediated postsynaptic translation and mRNA metabolism.
Pathological manifestation of human endogenous retrovirus K in frontotemporal dementia.
Impact of a frequent nearsplice SOD1 variant in amyotrophic lateral sclerosis: optimising SOD1 genetic screening for gene therapy opportunities.
Riluzole does not ameliorate disease caused by cytoplasmic TDP-43 in a mouse model of amyotrophic lateral sclerosis.
ATG14 and RB1CC1 play essential roles in maintaining muscle homeostasis.
TDP-43 mediates SREBF2-regulated gene expression required for oligodendrocyte myelination.
MATR3 F115C knock-in mice do not exhibit motor defects or neuropathological features of ALS.
TDP-43 condensation properties specify its RNA-binding and regulatory repertoire.
A microfluidic approach to rescue ALS motor neuron degeneration using rapamycin.
Fast Progression in Amyotrophic Lateral Sclerosis Is Associated With Greater TDP-43 Burden in Spinal Cord.
TMEM106B modifies TDP-43 pathology in human ALS brain and cell-based models of TDP-43 proteinopathy.
Establishment of chemically oligomerizable TAR DNA-binding protein-43 which mimics amyotrophic lateral sclerosis pathology in mammalian cells.
Ribonuclease recruitment using a small molecule reduced c9ALS/FTD r(G(4)C(2)) repeat expansion in vitro and in vivo ALS models.
Transmission of amyloid-beta and tau pathologies is associated with cognitive impairments in a primate.
FTLD Patient-Derived Fibroblasts Show Defective Mitochondrial Function and Accumulation of p62.
Phosphorylation regulates arginine-rich RNA-binding protein solubility and oligomerization.
Long non-coding RNA NEAT1_1 ameliorates TDP-43 toxicity in in vivo models of TDP-43 proteinopathy.
Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins.
Mid to late-life scores of depression in the cognitively healthy are associated with cognitive status and Alzheimer's disease pathology at death.
Mislocalisation of TDP-43 to the cytoplasm causes cortical hyperexcitability and reduced excitatory neurotransmission in the motor cortex.
Globular glial tauopathy with a mutation in MAPT and unusual TDP-43 proteinopathy in a patient with behavioural-variant frontotemporal dementia.
Patterns of neuronal Rhes as a novel hallmark of tauopathies.
Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology.
FUS/TLS Suppresses Enterovirus Replication and Promotes Antiviral Innate Immune Responses.
Characterization of the human TARDBP gene promoter.
Genetic screening of ANXA11 revealed novel mutations linked to amyotrophic lateral sclerosis.
Identification of SRSF10 as a regulator of SMN2 ISS-N1.
A novel temporal-predominant neuro-astroglial tauopathy associated with TMEM106B gene polymorphism in FTLD/ALS-TDP.
Pontine Arteriolosclerosis and Locus Coeruleus Oxidative Stress Differentiate Resilience from Mild Cognitive Impairment in a Clinical Pathologic Cohort.
Hippocampal Sclerosis in Frontotemporal Dementia: When Vascular Pathology Meets Neurodegeneration.
Identification of Genetic Modifiers of TDP-43: Inflammatory Activation of Astrocytes for Neuroinflammation.
The E50K optineurin mutation impacts autophagy-mediated degradation of TDP-43 and leads to RGC apoptosis in vivo and in vitro.
Multiple Sclerosis-Associated hnRNPA1 Mutations Alter hnRNPA1 Dynamics and Influence Stress Granule Formation.
Deferoxamine mesylate improves splicing and GAA activity of the common c.-32-13T>G allele in late-onset PD patient fibroblasts.
TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells.
Staufen1 in Human Neurodegeneration.
MIR-NATs repress MAPT translation and aid proteostasis in neurodegeneration.
Expression of C9orf72 hexanucleotide repeat expansion leads to formation of RNA foci and dipeptide repeat proteins but does not influence autophagy or proteasomal function in neuronal cells.
O-GlcNAcylation of TDP-43 suppresses proteinopathies and promotes TDP-43's mRNA splicing activity.
Mouse closed head traumatic brain injury replicates the histological tau pathology pattern of human disease: characterization of a novel model and systematic review of the literature.
UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model.
Identification of hub molecules of FUS-ALS by Bayesian gene regulatory network analysis of iPSC model: iBRN.
Stimulation of mTOR-independent autophagy and mitophagy by rilmenidine exacerbates the phenotype of transgenic TDP-43 mice.
Effects of TDP-43 overexpression on neuron proteome and morphology in vitro.
Rapid in vitro quantification of TDP-43 and FUS mislocalisation for screening of gene variants implicated in frontotemporal dementia and amyotrophic lateral sclerosis.
Neuronal VCP loss of function recapitulates FTLD-TDP pathology.
CRISPR/Cas9-engineered Drosophila knock-in models to study VCP diseases.
Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43(G298S) transgenic mice.
Optineurin defects cause TDP43-pathology with autophagic vacuolar formation.
Active lifestyle enhances protein expression profile in subjects with Lewy body pathology.
Amyloid β and tau pathology in brains of aged pinniped species (sea lion, seal, and walrus).
The FUS gene is dual-coding with both proteins contributing to FUS-mediated toxicity.
Novel tankyrase inhibitors suppress TDP-43 aggregate formation.
Targeting nuclear protein TDP-43 by cell division cycle kinase 7 inhibitors: A new therapeutic approach for amyotrophic lateral sclerosis.
Small molecule targeting r(UGGAA)(n) disrupts RNA foci and alleviates disease phenotype in Drosophila model.
Clinicopathological Staging of Dynamics of Neurodegeneration and Neuronal Loss in Alzheimer Disease.
Repetitive Traumatic Brain Injury Is Associated With TDP-43 Alterations, Neurodegeneration, and Glial Activation in Mice.
Multiple system aging-related tau astrogliopathy with complex proteinopathy in an oligosymptomatic octogenarian.
TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS.
Therapeutic potential of novel Cell Division Cycle Kinase 7 inhibitors on TDP-43-related pathogenesis such as Frontotemporal Lobar Degeneration (FTLD) and amyotrophic lateral sclerosis (ALS).
A novel recessive mutation affecting DNAJB6a causes myofibrillar myopathy.
Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis.
The Periodontium Damage Induces Neuronal Cell Death in the Trigeminal Mesencephalic Nucleus and Neurodegeneration in the Trigeminal Motor Nucleus in C57BL/6J Mice.
PCBP2 Is Downregulated in Degenerating Neurons and Rarely Observed in TDP-43-Positive Inclusions in Sporadic Amyotrophic Lateral Sclerosis.
18F-AV-1451 positron emission tomography in neuropathological substrates of corticobasal syndrome.
TDP-43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann-Pick C disease.
Tau-tubulin kinase 1 phosphorylates TDP-43 at disease-relevant sites and exacerbates TDP-43 pathology.
Thioridazine reverts the phenotype in cellular and Drosophila models of amyotrophic lateral sclerosis by enhancing TDP-43 aggregate clearance.
Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.
NEAT1 is essential for metabolic changes that promote breast cancer growth and metastasis.
Defective cyclophilin A induces TDP-43 proteinopathy: implications for amyotrophic lateral sclerosis and frontotemporal dementia.
CellFIE: CRISPR- and Cell Fusion-based Two-hybrid Interaction Mapping of Endogenous Proteins.
Circ-Hdgfrp3 shuttles along neurites and is trapped in aggregates formed by ALS-associated mutant FUS.
TDP-43 stabilizes G3BP1 mRNA: relevance to amyotrophic lateral sclerosis/frontotemporal dementia.
Novel chemical inhibitor against SOD1 misfolding and aggregation protects neuron-loss and ameliorates disease symptoms in ALS mouse model.
Drivers of Hsp104 potentiation revealed by scanning mutagenesis of the middle domain.
Phosphorylation of trans-active response DNA-binding protein-of 43 kDa promotes its cytoplasmic aggregation and modulates its function in tau mRNA stability and exon 10 alternative splicing.
Clinical and neuropathological diversity of tauopathy in MAPT duplication carriers.
Tideglusib, a Non-ATP Competitive Inhibitor of GSK-3β as a Drug Candidate for the Treatment of Amyotrophic Lateral Sclerosis.
Haploinsufficiency of the TDP43 ubiquitin E3 ligase RNF220 leads to ALS-like motor neuron defects in the mouse.
Extensive phenotypic characterisation of a human TDP-43(Q331K) transgenic mouse model of amyotrophic lateral sclerosis (ALS).
A ligand-insensitive UNC5B splicing isoform regulates angiogenesis by promoting apoptosis.
Decoding distinctive features of plasma extracellular vesicles in amyotrophic lateral sclerosis.
Targeting TDP-43 Pathology Alleviates Cognitive and Motor Deficits Caused by Chronic Cerebral Hypoperfusion.
Enhanced detection of expanded repeat mRNA foci with hybridization chain reaction.
Characterization of the upstream and intron promoters of the gene encoding TAR DNA-binding protein.
TDP-43 prevents endogenous RNAs from triggering a lethal RIG-I-dependent interferon response.
Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice.
The Role of HDAC6 in TDP-43-Induced Neurotoxicity and UPS Impairment.
Pathological Correlations of Neuropsychiatric Symptoms in Institutionalized People with Dementia.
The Contribution of Vascular Pathology Toward Cognitive Impairment in Older Individuals with Intermediate Braak Stage Tau Pathology.
TDP-43 Related Neuropathologies and Phosphorylation State: Associations with Age and Clinical Dementia in the Cambridge City over-75s Cohort.
A Comparative Study of Pathological Outcomes in The University of Manchester Longitudinal Study of Cognition in Normal Healthy Old Age and Brains for Dementia Research Cohorts.
Functional analysis of proposed substrate-binding residues of Hsp104.
Neurodegeneration-Associated Proteins in Human Olfactory Neurons Collected by Nasal Brushing.
Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation.
Skeletal Muscle-Restricted Expression of Human SOD1 in Transgenic Mice Causes a Fatal ALS-Like Syndrome.
BV-2 Microglial Cells Overexpressing C9orf72 Hexanucleotide Repeat Expansion Produce DPR Proteins and Show Normal Functionality but No RNA Foci.
Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity.
Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations.
Isolation and characterization of antibody fragments selective for human FTD brain derived TDP-43 variants.
Conditioned Medium from Cells Overexpressing TDP-43 Alters the Metabolome of Recipient Cells.
TDP-43 mediated blood-brain barrier permeability and leukocyte infiltration promote neurodegeneration in a low-grade systemic inflammation mouse model.
Stress Granule Assembly Can Facilitate but Is Not Required for TDP-43 Cytoplasmic Aggregation.
G(4)C(2) Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD.
The effect and mechanism of 19S proteasome PSMD11/Rpn6 subunit in D-Galactose induced mimetic aging models.
Absence of Subcerebral Projection Neurons Is Beneficial in a Mouse Model of Amyotrophic Lateral Sclerosis.
Cytoplasmic localization of amyotrophic lateral sclerosis-related TDP-43 proteins modulates stress granule formation.
Nucleated transcriptional condensates amplify gene expression.
An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress.
The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B-mediated inflammatory response.
Spinal Cord and Motor Neuron TDP-43 Pathology in a Sporadic Inclusion Body Myositis Patient.
Sensitivity-Specificity of Tau and Amyloid β Positron Emission Tomography in Frontotemporal Lobar Degeneration.
Multiplexed chemogenetics in astrocytes and motoneurons restore blood-spinal cord barrier in ALS.
Cytoplasmic mislocalization and mitochondrial colocalization of TDP-43 are common features between normal aged and young mice.
TDP-43 pathology in primary lateral sclerosis.
Cognitive deficit, and neuropathological correlates, in the oldest-old.
Chronic stress induces formation of stress granules and pathological TDP-43 aggregates in human ALS fibroblasts and iPSC-motoneurons.
TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons.
Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD-TDP subtypes.
TDP-43 interacts with amyloid-β, inhibits fibrillization, and worsens pathology in a model of Alzheimer's disease.
Loss of TDP-43 in astrocytes leads to motor deficits by triggering A1-like reactive phenotype and triglial dysfunction.
TDP-43 proteinopathies: a new wave of neurodegenerative diseases.
Novel monoclonal antibodies targeting the RRM2 domain of human TDP-43 protein.
RNA-binding protein altered expression and mislocalization in MS.
Mutant C9orf72 human iPSC-derived astrocytes cause non-cell autonomous motor neuron pathophysiology.
ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation and a higher susceptibility to DNA damage.
Transmission of ALS pathogenesis by the cerebrospinal fluid.
HDAC1 inhibition ameliorates TDP-43-induced cell death in vitro and in vivo.
A role for SUMOylation in the Formation and Cellular Localization of TDP-43 Aggregates in Amyotrophic Lateral Sclerosis.
Elevation of casein kinase 1ε associated with TDP-43 and tau pathologies in Alzheimer's disease.
Sequestosome-1 (p62) expression reveals chaperone-assisted selective autophagy in immune-mediated necrotizing myopathies.
Forced isoform switching of Neat1_1 to Neat1_2 leads to the loss of Neat1_1 and the hyperformation of paraspeckles but does not affect the development and growth of mice.
Human subiculo-fornico-mamillary system in Alzheimer's disease: Tau seeding by the pillar of the fornix.
The SINEB1 element in the long non-coding RNA Malat1 is necessary for TDP-43 proteostasis.
Sigma-1 receptor is a key genetic modulator in amyotrophic lateral sclerosis.
Motor neuron preservation and decrease of in vivo TDP-43 phosphorylation by protein CK-1δ kinase inhibitor treatment.
CYLD is a causative gene for frontotemporal dementia - amyotrophic lateral sclerosis.
Clinical and pathologic phenotype of a large family with heterozygous STUB1 mutation.
Mutant TDP-43 Expression Triggers TDP-43 Pathology and Cell Autonomous Effects on Primary Astrocytes: Implications for Non-cell Autonomous Pathology in ALS.
Endoplasmic Reticulum Stress Signalling Induces Casein Kinase 1-Dependent Formation of Cytosolic TDP-43 Inclusions in Motor Neuron-Like Cells.
Diagnostic and prognostic values of PBMC proteins in amyotrophic lateral sclerosis.
HDAC1 dysregulation induces aberrant cell cycle and DNA damage in progress of TDP-43 proteinopathies.
RBM45 associates with nuclear stress bodies and forms nuclear inclusions during chronic cellular stress and in neurodegenerative diseases.
Mitigation of ALS Pathology by Neuron-Specific Inhibition of Nuclear Factor Kappa B Signaling.
TDP-43, a protein central to amyotrophic lateral sclerosis, is destabilized by tankyrase-1 and -2.
Extracellular Vesicles Isolated from Human Induced Pluripotent Stem Cell-Derived Neurons Contain a Transcriptional Network.
SOD1 p.D12Y variant is associated with amyotrophic lateral sclerosis/distal myopathy spectrum.
Amyotrophic Lateral Sclerosis Modifiers in Drosophila Reveal the Phospholipase D Pathway as a Potential Therapeutic Target.
The Impact of ALS-Associated Genes hnRNPA1, MATR3, VCP and UBQLN2 on the Severity of TDP-43 Aggregation.
DeepCLIP: predicting the effect of mutations on protein-RNA binding with deep learning.
Modeling of Frontotemporal Dementia Using iPSC Technology.
Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function.
Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36.
Revealing the Proteome of Motor Cortex Derived Extracellular Vesicles Isolated from Amyotrophic Lateral Sclerosis Human Postmortem Tissues.
Glutathione S-Transferase Rescues Motor Neuronal Toxicity in Fly Model of Amyotrophic Lateral Sclerosis.
Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS).
Subcortical TDP-43 pathology patterns validate cortical FTLD-TDP subtypes and demonstrate unique aspects of C9orf72 mutation cases.
Overexpression of human wtTDP-43 causes impairment in hippocampal plasticity and behavioral deficits in CAMKII-tTa transgenic mouse model.
Cdc48/VCP and Endocytosis Regulate TDP-43 and FUS Toxicity and Turnover.
Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage.
A robust TDP-43 knock-in mouse model of ALS.
Multiple distinct pathways lead to hyperubiquitylated insoluble TDP-43 protein independent of its translocation into stress granules.
TDP-43 facilitates milk lipid secretion by post-transcriptional regulation of Btn1a1 and Xdh.
Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting.
Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model.
Use of Capillary Electrophoresis Immunoassay to Search for Potential Biomarkers of Amyotrophic Lateral Sclerosis in Human Platelets.
From the Entorhinal Region via the Prosubiculum to the Dentate Fascia: Alzheimer Disease-Related Neurofibrillary Changes in the Temporal Allocortex.
Neurotoxic microglia promote TDP-43 proteinopathy in progranulin deficiency.
An Experimental Model of Neurodegenerative Disease Based on Porcine Hemagglutinating Encephalomyelitis Virus-Related Lysosomal Abnormalities.
Accumulation of phosphorylated TDP-43 in the cytoplasm of Schwann cells in a case of sporadic amyotrophic lateral sclerosis.
LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient.
TDP-43 mutations link Amyotrophic Lateral Sclerosis with R-loop homeostasis and R loop-mediated DNA damage.
Optogenetic TDP-43 nucleation induces persistent insoluble species and progressive motor dysfunction in vivo.
Influence of APOE genotype in primary age-related tauopathy.
Analysis of ALS-related proteins during herpes simplex virus-2 latent infection.
18F-flortaucipir PET to autopsy comparisons in Alzheimer's disease and other neurodegenerative diseases.
Evidence of A Negative Feedback Network Between TDP-43 and miRNAs Dependent on TDP-43 Nuclear Localization.
Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions.
Calpain-1 and Calpain-2 in the Brain: New Evidence for a Critical Role of Calpain-2 in Neuronal Death.
Therapeutic genetic variation revealed in diverse Hsp104 homologs.
Cytosolic calcium regulates cytoplasmic accumulation of TDP-43 through Calpain-A and Importin α3.
The vulnerability of motor and frontal cortex-dependent behaviors in mice expressing ALS-linked mutation in TDP-43.
A New Glycogen Storage Disease Caused by a Dominant PYGM Mutation.
All-Trans Retinoic Acid Exerts Neuroprotective Effects in Amyotrophic Lateral Sclerosis-Like Tg (SOD1*G93A)1Gur Mice.
PTK2/FAK regulates UPS impairment via SQSTM1/p62 phosphorylation in TARDBP/TDP-43 proteinopathies.
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation.
Multiple system atrophy pathology is associated with primary Sjögren's syndrome.
Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy.
Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains.
Reprogramming fibroblasts and peripheral blood cells from a C9ORF72 patient: A proof-of-principle study.
Upregulation of ATG7 attenuates motor neuron dysfunction associated with depletion of TARDBP/TDP-43.
The Amyloid Inhibitor CLR01 Relieves Autophagy and Ameliorates Neuropathology in a Severe Lysosomal Storage Disease.
Astrocytes with TDP-43 inclusions exhibit reduced noradrenergic cAMP and Ca(2+) signaling and dysregulated cell metabolism.
The cellular expression and proteolytic processing of the amyloid precursor protein is independent of TDP-43.
Distinct molecular patterns of TDP-43 pathology in Alzheimer's disease: relationship with clinical phenotypes.
Cell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD.
Fabry Disease With Concomitant Lewy Body Disease.
Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis.
Inactive USP14 and inactive UCHL5 cause accumulation of distinct ubiquitinated proteins in mammalian cells.
Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration.
Developmental Expression of Mutant PFN1 in Motor Neurons Impacts Neuronal Growth and Motor Performance of Young and Adult Mice.
Repeated Low-Level Blast Overpressure Leads to Endovascular Disruption and Alterations in TDP-43 and Piezo2 in a Rat Model of Blast TBI.
RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether.
Myelin degeneration induced by mutant superoxide dismutase 1 accumulation promotes amyotrophic lateral sclerosis.
Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)-binding protein 1 in Huntington's disease.
TDP-43 regulates transcription at protein-coding genes and Alu retrotransposons.
Non-genetically modified models exhibit TARDBP mRNA increase due to perturbed TDP-43 autoregulation.
Neuronal TDP-43 depletion affects activity-dependent plasticity.
Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia.
Phosphorylated TDP-43 aggregates in skeletal and cardiac muscle are a marker of myogenic degeneration in amyotrophic lateral sclerosis and various conditions.
Phase separation-deficient TDP43 remains functional in splicing.
Design, synthesis, and biological evaluation of novel tetrahydroprotoberberine derivatives (THPBs) as proprotein convertase subtilisin/kexin type 9 (PCSK9) modulators for the treatment of hyperlipidemia.
Neuropathological lesions in the very old: results from a large Brazilian autopsy study.
Clinicopathologic correlations in a family with a TBK1 mutation presenting as primary progressive aphasia and primary lateral sclerosis.
Splicing repression is a major function of TDP-43 in motor neurons.
Increased prevalence of granulovacuolar degeneration in C9orf72 mutation.
Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo.
Memory Decline and Its Reversal in Aging and Neurodegeneration Involve miR-183/96/182 Biogenesis.
Dysregulation of TDP-43 intracellular localization and early onset ALS are associated with a TARDBP S375G variant.
TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response.
Proteomic analysis reveals that wildtype and alanine-expanded nuclear poly(A)-binding protein exhibit differential interactions in skeletal muscle.
ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97.
Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations.
PARylation regulates stress granule dynamics, phase separation, and neurotoxicity of disease-related RNA-binding proteins.
TDP-43 levels are higher in platelets from patients with sporadic amyotrophic lateral sclerosis than in healthy controls.
Aggregation of the nucleic acid-binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation.
Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events.
Biomarkers for diseases with TDP-43 pathology.
Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.
VCP maintains lysosomal homeostasis and TFEB activity in differentiated skeletal muscle.
Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition.
Association between TDP-43 and mitochondria in inclusion body myositis.
Autosomal dominant mitochondrial membrane protein-associated neurodegeneration (MPAN).
Neuronal Expression of UBQLN2(P497H) Exacerbates TDP-43 Pathology in TDP-43(G348C) Mice through Interaction with Ubiquitin.
TDP-43 regulates early-phase insulin secretion via CaV1.2-mediated exocytosis in islets.
C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation.
Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR.
Identification and therapeutic rescue of autophagosome and glutamate receptor defects in C9ORF72 and sporadic ALS neurons.
Persistent neuropathological effects 14 years following amyloid-β immunization in Alzheimer's disease.
Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1(G93A) Rodent Spinal Cord Tissue.
Development of an automated capillary nano-immunoassay-Simple Western assay-to quantify total TDP43 protein in human platelet samples.
TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates.
Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein.
Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology.
Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.
Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS.
(18)F-flortaucipir (AV-1451) tau PET in frontotemporal dementia syndromes.
Rasagiline for amyotrophic lateral sclerosis: A randomized, controlled trial.
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration.
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair.
Brain banking in low and middle-income countries: Raison D'être for the Ibadan Brain Ageing, Dementia And Neurodegeneration (IBADAN) Brain Bank Project.
Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology.
Virus-mediated delivery of antibody targeting TAR DNA-binding protein-43 mitigates associated neuropathology.
The Small Non-coding Vault RNA1-1 Acts as a Riboregulator of Autophagy.
Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis.
Physiologically Important Electrolytes as Regulators of TDP-43 Aggregation and Droplet-Phase Behavior.
Muscle pathology of hereditary motor and sensory neuropathy with proximal dominant involvement with TFG mutation.
The long noncoding RNA neuroLNC regulates presynaptic activity by interacting with the neurodegeneration-associated protein TDP-43.
Neuroglial patterns are shared by cerebella from prion and prion-like disorder affected patients.
TDP-43 knockdown causes innate immune activation via protein kinase R in astrocytes.
Luminescent conjugated oligothiophenes distinguish between α-synuclein assemblies of Parkinson's disease and multiple system atrophy.
Activity of the poly(A) binding protein MSUT2 determines susceptibility to pathological tau in the mammalian brain.
Nopp140-mediated concentration of telomerase in Cajal bodies regulates telomere length.
Compromised function of the ESCRT pathway promotes endolysosomal escape of tau seeds and propagation of tau aggregation.
Suppression of Progranulin Expression Leads to Formation of Intranuclear TDP-43 Inclusions In Vitro: A Cell Model of Frontotemporal Lobar Degeneration.
RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy.
Altered immunoreactivity of ErbB4, a causative gene product for ALS19, in the spinal cord of patients with sporadic ALS.
Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity.
CYLD dysregulation in pathogenesis of sporadic inclusion body myositis.
Amyotrophic lateral sclerosis-associated TDP-43 mutation Q331K prevents nuclear translocation of XRCC4-DNA ligase 4 complex and is linked to genome damage-mediated neuronal apoptosis.
Recapitulation of Pathological TDP-43 Features in Immortalized Lymphocytes from Sporadic ALS Patients.
Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS.
Loss of Nuclear TDP-43 Is Associated with Decondensation of LINE Retrotransposons.
Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice.
Drosophila Ref1/ALYREF regulates transcription and toxicity associated with ALS/FTD disease etiologies.
Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation.
Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway.
Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death.
RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43.
Properties of LINE-1 proteins and repeat element expression in the context of amyotrophic lateral sclerosis.
Myopathy With SQSTM1 and TIA1 Variants: Clinical and Pathological Features.
Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice.
Cryptic exon splicing function of TARDBP interacts with autophagy in nervous tissue.
mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms.
DGCR8 Promotes Neural Progenitor Expansion and Represses Neurogenesis in the Mouse Embryonic Neocortex.
Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration.
Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.
TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging.
Distinct multilevel misregulations of Parkin and PINK1 revealed in cell and animal models of TDP-43 proteinopathy.
FTLD/ALS-linked TDP-43 mutations do not alter TDP-43's ability to self-regulate its expression in Drosophila.
Spreading of Tau Pathology in Sporadic Alzheimer's Disease Along Cortico-cortical Top-Down Connections.
Towards a TDP-43-Based Biomarker for ALS and FTLD.
Colocalization of Bunina bodies and TDP-43 inclusions in a case of sporadic amyotrophic lateral sclerosis with Lewy body-like hyaline inclusions.
Subarachnoid hemorrhage enhances the expression of TDP-43 in the brain of experimental rats and human subjects.
Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.
Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity.
Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice.
Epigenetic Changes Associated with the Expression of Amyotrophic Lateral Sclerosis (ALS) Causing Genes.
Identification and characterization of ubiquitinylation sites in TAR DNA-binding protein of 43 kDa (TDP-43).
TAR DNA-Binding Protein 43 and Disrupted in Schizophrenia 1 Coaggregation Disrupts Dendritic Local Translation and Mental Function in Frontotemporal Lobar Degeneration.
TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle.
Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions.
Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage.
Mutant UBQLN2(P497H) in motor neurons leads to ALS-like phenotypes and defective autophagy in rats.
Liquid Nuclear Condensates Mechanically Sense and Restructure the Genome.
Angiopoietin-1 and ανβ3 integrin peptide promote the therapeutic effects of L-serine in an amyotrophic lateral sclerosis/Parkinsonism dementia complex model.
Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization.
TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types.
Essential role of hippocampal noradrenaline in the regulation of spatial working memory and TDP-43 tissue pathology.
TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis.
Overlapping Role of SCYL1 and SCYL3 in Maintaining Motor Neuron Viability.
Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms.
Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay.
TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.
Zc3h13 Regulates Nuclear RNA m(6)A Methylation and Mouse Embryonic Stem Cell Self-Renewal.
A single N-terminal phosphomimic disrupts TDP-43 polymerization, phase separation, and RNA splicing.
DCTN1 F52L mutation case of Perry syndrome with progressive supranuclear palsy-like tauopathy.
Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.
Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice.
Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.
Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis.
Motor neuron differentiation of iPSCs obtained from peripheral blood of a mutant TARDBP ALS patient.
A patient with posterior cortical atrophy due to Alzheimer's disease.
The age of onset and evolution of Braak tangle stage and Thal amyloid pathology of Alzheimer's disease in individuals with Down syndrome.
Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism.
Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease.
Alzheimer neuropathology without frontotemporal lobar degeneration hallmarks (TAR DNA-binding protein 43 inclusions) in missense progranulin mutation Cys139Arg.
A proteomic network approach across the ALS-FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain.
Characterization of Dopaminergic System in the Striatum of Young Adult Park2(-/-) Knockout Rats.
Mutation-dependent aggregation and toxicity in a Drosophila model for UBQLN2-associated ALS.
Anterior Cingulate Cortex TDP-43 Pathology in Sporadic Amyotrophic Lateral Sclerosis.
Repetitive Mild Closed Head Injury Alters Protein Expression and Dendritic Complexity in a Mouse Model.
Protein Phosphatase 1 dephosphorylates TDP-43 and suppresses its function in tau exon 10 inclusion.
Angiogenin Prevents Progranulin A9D Mutation-Induced Neuronal-Like Cell Apoptosis Through Cleaving tRNAs into tiRNAs.
Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons.
Cerebral ischemia induces the aggregation of proteins linked to neurodegenerative diseases.
Citrullination of RGG Motifs in FET Proteins by PAD4 Regulates Protein Aggregation and ALS Susceptibility.
CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency.
Behavioral defects in a DCTN1(G71A) transgenic mouse model of Perry syndrome.
Multiple Proteinopathies in Familial ALS Cases With Optineurin Mutations.
Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.
TDP-43 pathology in multiple system atrophy: colocalization of TDP-43 and α-synuclein in glial cytoplasmic inclusions.
Enteroviral Infection Leads to Transactive Response DNA-Binding Protein 43 Pathology in Vivo.
Transcription-Dependent Formation of Nuclear Granules Containing FUS and RNA Pol II.
Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies.
Identification of TCERG1 as a new genetic modulator of TDP-43 production in Drosophila.
Primary fibroblasts derived from sporadic amyotrophic lateral sclerosis patients do not show ALS cytological lesions.
Analysis of endocannabinoid receptors and enzymes in the post-mortem motor cortex and spinal cord of amyotrophic lateral sclerosis patients.
Disruption by SaCas9 Endonuclease of HERV-Kenv, a Retroviral Gene with Oncogenic and Neuropathogenic Potential, Inhibits Molecules Involved in Cancer and Amyotrophic Lateral Sclerosis.
Transactive Response DNA-Binding Protein 43 Abnormalities after Traumatic Brain Injury.
Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain.
RNA and Protein Interactors with TDP-43 in Human Spinal-Cord Lysates in Amyotrophic Lateral Sclerosis.
Neurodegenerative Disease Proteinopathies Are Connected to Distinct Histone Post-translational Modification Landscapes.
Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis.
Human Endogenous Retrovirus-K and TDP-43 Expression Bridges ALS and HIV Neuropathology.
Diabetes is Not Associated with Alzheimer's Disease Neuropathology.
Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy.
Tau Rather than TDP-43 Proteins are Potential Cerebrospinal Fluid Biomarkers for Frontotemporal Lobar Degeneration Subtypes: A Pilot Study.
Total proteome turbidity assay for tracking global protein aggregation in the natural cellular environment.
Regional correlations between [(11)C]PIB PET and post-mortem burden of amyloid-beta pathology in a diverse neuropathological cohort.
Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant.
Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS.
Decreased Levels of Foldase and Chaperone Proteins Are Associated with an Early-Onset Amyotrophic Lateral Sclerosis.
Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.
Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity.
Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease.
Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Traumatic Brain Injury Results in Cellular, Structural and Functional Changes Resembling Motor Neuron Disease.
Splicing factors act as genetic modulators of TDP-43 production in a new autoregulatory TDP-43 Drosophila model.
(18) F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: A multicenter study.
The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization.
Association between diabetes and causes of dementia: Evidence from a clinicopathological study.
Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43(A315T) ALS/FTD mouse model.
Identification of functional tetramolecular RNA G-quadruplexes derived from transfer RNAs.
Novel miR-b2122 regulates several ALS-related RNA-binding proteins.
Cis P-tau is induced in clinical and preclinical brain injury and contributes to post-injury sequelae.
Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing.
Platelet phosphorylated TDP-43: an exploratory study for a peripheral surrogate biomarker development for Alzheimer's disease.
Clusterin protects neurons against intracellular proteotoxicity.
TDP-43 accelerates age-dependent degeneration of interneurons.
TDP-43 misexpression causes defects in dendritic growth.
Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.
Incipient progressive supranuclear palsy is more common than expected and may comprise clinicopathological subtypes: a forensic autopsy series.
Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.
Dioxins and related environmental contaminants increase TDP-43 levels.
Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Clinical Significance of TDP-43 Neuropathology in Amyotrophic Lateral Sclerosis.
Absence of Alzheimer Disease Neuropathologic Changes in Eyes of Subjects With Alzheimer Disease.
TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway.
Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes.
Neuropathological diagnoses and clinical correlates in older adults in Brazil: A cross-sectional study.
DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy.
Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS.
Single cell imaging and quantification of TDP-43 and α-synuclein intercellular propagation.
Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function.
Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants.
Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons.
Simple Derivation of Spinal Motor Neurons from ESCs/iPSCs Using Sendai Virus Vectors.
C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.
Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies.
Cryptic exon incorporation occurs in Alzheimer's brain lacking TDP-43 inclusion but exhibiting nuclear clearance of TDP-43.
Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration.
Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation.
Dcp1a and GW182 Induce Distinct Cellular Aggregates and Have Different Effects on microRNA Pathway.
Misfolded SOD1 is not a primary component of sporadic ALS.
Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration.
TDP-43 expression influences amyloidβ plaque deposition and tau aggregation.
Anti-AMPA GluA3 antibodies in Frontotemporal dementia: a new molecular target.
Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells.
TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss.
Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program.
Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions.
Pathological correlations of [F-18]-AV-1451 imaging in non-alzheimer tauopathies.
Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis.
Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.
A Multi-network Approach Identifies Protein-Specific Co-expression in Asymptomatic and Symptomatic Alzheimer's Disease.
TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis.
Imaging specific newly synthesized proteins within cells by fluorescence resonance energy transfer.
Impaired activation of ALS monocytes by exosomes.
Tdp-43 cryptic exons are highly variable between cell types.
An intronic variation in SLC52A1 causes exon skipping and transient riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
Imbalanced expression of polycistronic miRNA in acute myeloid leukemia.
Clinical and neuropathological features of ALS/FTD with TIA1 mutations.
De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies.
TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling.
TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents.
Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4.
Endocytosis regulates TDP-43 toxicity and turnover.
GPNMB ameliorates mutant TDP-43-induced motor neuron cell death.
Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle.
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics.
Perry Syndrome: A Distinctive Type of TDP-43 Proteinopathy.
VEGF alleviates ALS-CSF induced cytoplasmic accumulations of TDP-43 and FUS/TLS in NSC-34 cells.
Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.
Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene.
Multireceptor fingerprints in progressive supranuclear palsy.
Increased cytoplasmic TDP-43 reduces global protein synthesis by interacting with RACK1 on polyribosomes.
Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables.
Progression of tau pathology within cholinergic nucleus basalis neurons in chronic traumatic encephalopathy: A chronic effects of neurotrauma consortium study.
Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia.
DYT6 Dystonia: A Neuropathological Study.
Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules.
Detection of hyperphosphorylated tau protein and α-synuclein in spinal cord of patients with Alzheimer's disease.
Distinct Muscle Biopsy Findings in Genetically Defined Adult-Onset Motor Neuron Disorders.
Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome.
Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition.
TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins.
Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization.
Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice.
Immunoprecipitation and mass spectrometry defines an extensive RBM45 protein-protein interaction network.
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo.
Level of PICALM, a key component of clathrin-mediated endocytosis, is correlated with levels of phosphotau and autophagy-related proteins and is associated with tau inclusions in AD, PSP and Pick disease.
Axonal TDP-43 aggregates in sporadic amyotrophic lateral sclerosis.
GRN and MAPT Mutations in 2 Frontotemporal Dementia Research Centers in Brazil.
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
Reduced stress granule formation and cell death in fibroblasts with the A382T mutation of TARDBP gene: evidence for loss of TDP-43 nuclear function.
Role of BMP receptor traffic in synaptic growth defects in an ALS model.
Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis.
Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43.
Unusual Processing Generates SPA LncRNAs that Sequester Multiple RNA Binding Proteins.
Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons.
The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis.
Analysis of RNA metabolism in peripheral WBCs of TDP-43 KI mice identifies novel biomarkers of ALS.
Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs.
Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C.
Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation.
T-Cell-Mediated Inflammatory Myopathies in HIV-Positive Individuals: A Histologic Study of 19 Cases.
Mechanistic Insights into Hsp104 Potentiation.
Loss of VPS13C Function in Autosomal-Recessive Parkinsonism Causes Mitochondrial Dysfunction and Increases PINK1/Parkin-Dependent Mitophagy.
G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunits.
C9orf72 is required for proper macrophage and microglial function in mice.
Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains.
Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43.
Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1C2-positive polyglutamine inclusions.
An autopsied case of unclassifiable sporadic four-repeat tauopathy presenting with parkinsonism and speech disturbances.
Defining the spectrum of frontotemporal dementias associated with TARDBP mutations.
Misregulation of Alternative Splicing in a Mouse Model of Rett Syndrome.
Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD.
Phenotypic characteristics of Alzheimer patients carrying an ABCA7 mutation.
The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice.
Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes.
A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects.
Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study.
Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43.
Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.
Global identification of hnRNP A1 binding sites for SSO-based splicing modulation.
Chronic Traumatic Encephalopathy Presenting as Alzheimer's Disease in a Retired Soccer Player.
Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.
MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.
Mutations in HSPB8 causing a new phenotype of distal myopathy and motor neuropathy.
TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion.
CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.
Intravenous injection of l-BMAA induces a rat model with comprehensive characteristics of amyotrophic lateral sclerosis/Parkinson-dementia complex.
An autopsy case of frontotemporal lobar degeneration with the appearance of fused in sarcoma inclusions (basophilic inclusion body disease) clinically presenting corticobasal syndrome.
Clinicopathological description of two cases with SQSTM1 gene mutation associated with frontotemporal dementia.
Clinical features of TBK1 carriers compared with C9orf72, GRN and non-mutation carriers in a Belgian cohort.
Serine 403-phosphorylated p62/SQSTM1 immunoreactivity in inclusions of neurodegenerative diseases.
Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D.
Melatonin enhances arsenic trioxide-induced cell death via sustained upregulation of Redd1 expression in breast cancer cells.
In vitro prion-like behaviour of TDP-43 in ALS.
Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia.
Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model.
Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis.
Nuclear bodies reorganize during myogenesis in vitro and are differentially disrupted by expression of FSHD-associated DUX4.
Marked Involvement of the Striatal Efferent System in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity.
Severe muscle wasting and denervation in mice lacking the RNA-binding protein ZFP106.
A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia.
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Multiple sclerosis masquerading as Alzheimer-type dementia: Clinical, radiological and pathological findings.
Targeting TDP-43 phosphorylation by Casein Kinase-1δ inhibitors: a novel strategy for the treatment of frontotemporal dementia.
ESCRT-0 dysfunction compromises autophagic degradation of protein aggregates and facilitates ER stress-mediated neurodegeneration via apoptotic and necroptotic pathways.
Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils.
ALS patients with ability to communicate after long-term mechanical ventilation have confined degeneration to the motor neuron system.
ALS-linked protein disulfide isomerase variants cause motor dysfunction.
Miro1 deficiency in amyotrophic lateral sclerosis.
Progressive supranuclear palsy in a family with TDP-43 pathology.
AAV9 supports wide-scale transduction of the CNS and TDP-43 disease modeling in adult rats.
Identification of linc-NeD125, a novel long non coding RNA that hosts miR-125b-1 and negatively controls proliferation of human neuroblastoma cells.
Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates.
Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets.
A novel triple repeat mutant tau transgenic model that mimics aspects of pick's disease and fronto-temporal tauopathies.
TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation.
Inflammation Induces TDP-43 Mislocalization and Aggregation.
Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion.
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure.
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.
Validating novel tau positron emission tomography tracer [F-18]-AV-1451 (T807) on postmortem brain tissue.
Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease.
Early maturation and distinct tau pathology in induced pluripotent stem cell-derived neurons from patients with MAPT mutations.
Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.
Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS.
TDP-43 is intercellularly transmitted across axon terminals.
Amyotrophic lateral sclerosis-associated mutant profilin 1 increases dendritic arborisation and spine formation in primary hippocampal neurons.
Neuropathologic features of suicide victims who presented with acute poststroke depression: significance of association with neurodegenerative disorders.
Analysis of β-N-methylamino-L-alanine (L-BMAA) neurotoxicity in rat cerebellum.
Primary fibroblasts cultures reveal TDP-43 abnormalities in amyotrophic lateral sclerosis patients with and without SOD1 mutations.
A Peruvian family with a novel PARK2 mutation: Clinical and pathological characteristics.
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia.
Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport.
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity.
Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.
Defining neurodegeneration on Guam by targeted genomic sequencing.
TARDBP pathogenic mutations increase cytoplasmic translocation of TDP-43 and cause reduction of endoplasmic reticulum Ca²⁺ signaling in motor neurons.
TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model.
Increasing progranulin levels and blockade of the ERK1/2 pathway: upstream and downstream strategies for the treatment of progranulin deficient frontotemporal dementia.
Pathogenic Ubqln2 gains toxic properties to induce neuron death.
Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43.
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs.
Hippocampal sclerosis in the parkinsonism-dementia complex of Guam: quantitative examination of neurons, neurofibrillary tangles, and TDP-43 immunoreactivity in CA1.
Minimal change multiple system atrophy: an aggressive variant?
Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.
Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations.
Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons.
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.
Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death.
Neuropathological assessments of the pathology in frontotemporal lobar degeneration with TDP43-positive inclusions: an inter-laboratory study by the BrainNet Europe consortium.
Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways.
Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy.
4-Hydroxynonenal induces persistent insolubilization of TDP-43 and alters its intracellular localization.
Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia.
Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A.
An acetylation switch controls TDP-43 function and aggregation propensity.
The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity.
TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain.
Changes in TDP-43 expression in development, aging, and in the neurofilament light protein knockout mouse.
Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy.
Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation.
Novel atomic force microscopy based biopanning for isolation of morphology specific reagents against TDP-43 variants in amyotrophic lateral sclerosis.
Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.
Primary chronic traumatic encephalopathy in an older patient with late-onset AD phenotype.
Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases.
From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models.
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.
C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
Disparate Mutations Confer Therapeutic Gain of Hsp104 Function.
An age-related reduction of brain TBPH/TDP-43 levels precedes the onset of locomotion defects in a Drosophila ALS model.
Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D.
R47H Variant of TREM2 Associated With Alzheimer Disease in a Large Late-Onset Family: Clinical, Genetic, and Neuropathological Study.
G1/S Cell Cycle Checkpoint Dysfunction in Lymphoblasts from Sporadic Parkinson's Disease Patients.
Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.
Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features.
Expanding the spectrum of neuronal pathology in multiple system atrophy.
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD.
Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis.
SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions.
Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2.
A phosphomimetic mutant TDP-43 (S409/410E) induces Drosha instability and cytotoxicity in Neuro 2A cells.
An optimized InCell Western screening technique identifies hexachlorophene as a novel potent TDP43 targeting drug.
Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates.
Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes.
A novel mutation P112H in the TARDBP gene associated with frontotemporal lobar degeneration without motor neuron disease and abundant neuritic amyloid plaques.
FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.
G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA.
TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodies.
Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dismutase-1 gene associated with amyotrophic lateral sclerosis.
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
Gene targeting of mouse Tardbp negatively affects Masp2 expression.
Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cells.
Casein kinase II induced polymerization of soluble TDP-43 into filaments is inhibited by heat shock proteins.
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
Th1 response and systemic treg deficiency in inclusion body myositis.
Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.
Increased metal content in the TDP-43(A315T) transgenic mouse model of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
The co-occurrence of Alzheimer's disease and Huntington's disease: a neuropathological study of 15 elderly Huntington's disease subjects.
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis.
TDP-43 regulates β-adducin (Add2) transcript stability.
Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury.
Atypical Alzheimer's disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons.
RNA metabolism in ALS: when normal processes become pathological.
Corticobasal degeneration initially developing motor versus non-motor symptoms: a comparative clinicopathological study.
TDP-43 toxicity proceeds via calcium dysregulation and necrosis in aging Caenorhabditis elegans motor neurons.
Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.
Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice.
Brain injection of α-synuclein induces multiple proteinopathies, gliosis, and a neuronal injury marker.
Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.
Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.
Hippocampal sclerosis dementia with the C9ORF72 hexanucleotide repeat expansion.
FTLD-ALS of TDP-43 type and SCA2 in a family with a full ataxin-2 polyglutamine expansion.
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response.
TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.
Concomitant accumulation of α-synuclein and TDP-43 in a patient with corticobasal degeneration.
ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress.
Intrathecal infusion of BMAA induces selective motor neuron damage and astrogliosis in the ventral horn of the spinal cord.
Neuropathological features of genetically confirmed DYT1 dystonia: investigating disease-specific inclusions.
CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome.
A novel GRN mutation (GRN c.708+6_+9delTGAG) in frontotemporal lobar degeneration with TDP-43-positive inclusions: clinicopathologic report of 6 cases.
Tar DNA-binding protein-43 (TDP-43) regulates axon growth in vitro and in vivo.
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis.
PRKAR1B mutation associated with a new neurodegenerative disorder with unique pathology.
Differential diagnosis of amyotrophic lateral sclerosis from Guillain-Barré syndrome by quantitative determination of TDP-43 in cerebrospinal fluid.
Astrocytic TDP-43 pathology in Alexander disease.
The RNA-binding protein TDP-43 selectively disrupts microRNA-1/206 incorporation into the RNA-induced silencing complex.
Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis.
Hereditary myopathy with early respiratory failure: occurrence in various populations.
Autopsy case of concurrent Huntington's disease and neurofibromatosis type 1.
Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disability.
Staging TDP-43 pathology in Alzheimer's disease.
ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arbor.
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species.
Plasma phosphorylated TDP-43 levels are elevated in patients with frontotemporal dementia carrying a C9orf72 repeat expansion or a GRN mutation.
Neuropathological features of multiple system atrophy with cognitive impairment.
Argyrophilic grain disease as a neurodegenerative substrate in late-onset schizophrenia and delusional disorders.
TAR DNA-binding protein 43 pathology in Alzheimer's disease with psychosis.
Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.
FUS is phosphorylated by DNA-PK and accumulates in the cytoplasm after DNA damage.
Mitochondrial dysfunction and decrease in body weight of transgenic knock-in mouse model for TDP-43: the question of glucose?
Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72.
Partial deletion of AFG3L2 causing spinocerebellar ataxia type 28.
Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis.
Neuropathologic assessment of dementia markers in identical and fraternal twins.
Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.
UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.
Myopathy-causing mutations in an HSP40 chaperone disrupt processing of specific client conformers.
RPL24: a potential therapeutic target whose depletion or acetylation inhibits polysome assembly and cancer cell growth.
Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice.
Metabolism and mis-metabolism of the neuropathological signature protein TDP-43.
Titinopathy in a Canadian family sharing the British founder haplotype.
Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice.
A high-content screen identifies novel compounds that inhibit stress-induced TDP-43 cellular aggregation and associated cytotoxicity.
Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf's neurons and other sacral motor neurons.
Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events.
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones.
De novo prion aggregates trigger autophagy in skeletal muscle.
Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.
Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation.
NF-Y inactivation causes atypical neurodegeneration characterized by ubiquitin and p62 accumulation and endoplasmic reticulum disorganization.
TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo.
No interaction between tau and TDP-43 pathologies in either frontotemporal lobar degeneration or motor neurone disease.
Cytoplasmic TDP-43 accumulation in cells of the adrenal medulla in individuals with or without amyotrophic lateral sclerosis.
H63D HFE genotype accelerates disease progression in animal models of amyotrophic lateral sclerosis.
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis.
An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution.
Aggregation properties of the small nuclear ribonucleoprotein U1-70K in Alzheimer disease.
Proteasome dysfunction induces muscle growth defects and protein aggregation.
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications.
The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.
Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.
FUS colocalizes with polyglutamine, but not with TDP-43 in neuronal intranuclear inclusions in spinocerebellar ataxia type 2.
Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases.
Ubiquitin-negative, eosinophilic neuronal cytoplasmic inclusions associated with stress granules and autophagy: an immunohistochemical investigation of two cases.
Reduced cellular Ca(2+) availability enhances TDP-43 cleavage by apoptotic caspases.
Parkin reverses TDP-43-induced cell death and failure of amino acid homeostasis.
A retrospective cohort study identifying the principal pathological features useful in the diagnosis of inclusion body myositis.
A novel approach for integrative studies on neurodegenerative diseases in human brains.
Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.
Diagnosis and management of behavioral variant frontotemporal dementia.
TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis.
Kinase Inhibitor Screening Identifies Cyclin-Dependent Kinases and Glycogen Synthase Kinase 3 as Potential Modulators of TDP-43 Cytosolic Accumulation during Cell Stress.
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
A 43-kDa TDP-43 species is present in aggregates associated with frontotemporal lobar degeneration.
Characterization of the distal polyadenylation site of the ß-adducin (Add2) pre-mRNA.
Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila.
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation.
Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.
Prevalence of dementia subtypes in a developing country: a clinicopathological study.
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.
β-N-methylamino-L-alanine induces changes in both GSK3 and TDP-43 in human neuroblastoma.
Clinicopathologic study of Alzheimer's disease: Alzheimer mimics.
ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease.
Neuropathology of partial PGC-1α deficiency recapitulates features of mitochondrial encephalopathies but not of neurodegenerative diseases.
Activation of transforming growth factor-β/Smad signaling reduces aggregate formation of mislocalized TAR DNA-binding protein-43.
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells.
β-N-methylamino-l-alanine causes neurological and pathological phenotypes mimicking Amyotrophic Lateral Sclerosis (ALS): the first step towards an experimental model for sporadic ALS.
Early and selective reduction of NOP56 (Asidan) and RNA processing proteins in the motor neuron of ALS model mice.
Mutation analysis and immunopathological studies of PFN1 in familial and sporadic amyotrophic lateral sclerosis.
Familial dementia with frontotemporal features associated with M146V presenilin-1 mutation.
Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene.
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons.
Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells.
Frontotemporal lobar degeneration: diversity of FTLD lesions.
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis.
Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2 delivery to motor neurons.
Amyotrophic lateral sclerosis: an update on recent genetic insights.
Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia.
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.
RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy.
C9ORF72 expansion in a family with bipolar disorder.
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
Extensive aggregation of α-synuclein and tau in juvenile-onset neuroaxonal dystrophy: an autopsied individual with a novel mutation in the PLA2G6 gene-splicing site.
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration.
Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43).
DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions.
Making the diagnosis of frontotemporal lobar degeneration.
The truncated C-terminal RNA recognition motif of TDP-43 protein plays a key role in forming proteinaceous aggregates.
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
Exome sequencing identifies titin mutations causing hereditary myopathy with early respiratory failure (HMERF) in families of diverse ethnic origins.
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.
Luminescent conjugated oligothiophenes for sensitive fluorescent assignment of protein inclusion bodies.
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration.
Progranulin mutations as risk factors for Alzheimer disease.
α-Synuclein coaggregation in familial amyotrophic lateral sclerosis with SOD1 gene mutation.
Optineurin is potentially associated with TDP-43 and involved in the pathogenesis of inclusion body myositis.
Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting.
Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolines.
Comment on "Drug screening for ALS using patient-specific induced pluripotent stem cells".
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function.
Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo.
Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis.
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.
The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis.
Neuron enriched nuclear proteome isolated from human brain.
The pathogenesis of cingulate atrophy in behavioral variant frontotemporal dementia and Alzheimer's disease.
Functional genomic screen of human stem cell differentiation reveals pathways involved in neurodevelopment and neurodegeneration.
Prion-like properties of pathological TDP-43 aggregates from diseased brains.
Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43.
Comparative utility of LC3, p62 and TDP-43 immunohistochemistry in differentiation of inclusion body myositis from polymyositis and related inflammatory myopathies.
Essential tremor followed by progressive supranuclear palsy: postmortem reports of 11 patients.
Expression of myogenic regulatory factors and myo-endothelial remodeling in sporadic inclusion body myositis.
African ancestry protects against Alzheimer's disease-related neuropathology.
Patterns of striatal degeneration in frontotemporal dementia.
Widespread aggregation of mutant VAPB associated with ALS does not cause motor neuron degeneration or modulate mutant SOD1 aggregation and toxicity in mice.
Glucocorticoids exacerbate cognitive deficits in TDP-25 transgenic mice via a glutathione-mediated mechanism: implications for aging, stress and TDP-43 proteinopathies.
New NBIA subtype: genetic, clinical, pathologic, and radiographic features of MPAN.
Involvement of endoplasmic reticulum stress in tauopathy.
Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes.
Regionally different immunoreactivity for Smurf2 and pSmad2/3 in TDP-43-positive inclusions of amyotrophic lateral sclerosis.
Pantothenate kinase-associated neurodegeneration is not a synucleinopathy.
A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: consideration of the pathology of post-polio syndrome.
Atypical FTLD-FUS associated with ALS-TDP: a case report.
Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.
Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis.
Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia.
Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6).
Identification of in vivo, conserved, TAF15 RNA binding sites reveals the impact of TAF15 on the neuronal transcriptome.
Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models.
The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function.
A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation.
A clinicopathological and genetic study of sporadic diffuse leukoencephalopathy with spheroids: a report of two cases.
Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
Increased number of astrocytes and macrophages/microglial cells in the corpus callosum in amyotrophic lateral sclerosis.
Altered astrocytic expression of TDP-43 does not influence motor neuron survival.
Neurocognitive speed associates with frontotemporal lobar degeneration TDP-43 subtypes.
Cell number changes in Alzheimer's disease relate to dementia, not to plaques and tangles.
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations.
Pathological features of FTLD-FUS in a Japanese population: analyses of nine cases.
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases.
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
Lack of unique neuropathology in amyotrophic lateral sclerosis associated with p.K54E angiogenin (ANG) mutation.
Surgical pathology of epilepsy-associated non-neoplastic cerebral lesions: a brief introduction with special reference to hippocampal sclerosis and focal cortical dysplasia.
Reducing TDP-43 aggregation does not prevent its cytotoxicity.
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.
G51D α-synuclein mutation causes a novel parkinsonian-pyramidal syndrome.
Welander distal myopathy caused by an ancient founder mutation in TIA1 associated with perturbed splicing.
Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons.
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS.
Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes.
Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio.
Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination.
TDP-43 identified from a genome wide RNAi screen for SOD1 regulators.
Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 tesla MRI and pathology.
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis.
Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans.
Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.
Clinical, neuropathological, and genetic characteristics of the novel IVS9+1delG GRN mutation in a patient with frontotemporal dementia.
Characterization of staufen1 ribonucleoproteins by mass spectrometry and biochemical analyses reveal the presence of diverse host proteins associated with human immunodeficiency virus type 1.
Lack of miRNA Misregulation at Early Pathological Stages in Drosophila Neurodegenerative Disease Models.
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology.
Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons.
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain.
FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation.
A novel TARDBP insertion/deletion mutation in the flail arm variant of amyotrophic lateral sclerosis.
TDP-43 plasma levels are higher in amyotrophic lateral sclerosis.
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
Benign hereditary chorea 2: pathological findings in an autopsy case.
An MND/ALS phenotype associated with C9orf72 repeat expansion: abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline.
In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer's disease.
Glucose delays age-dependent proteotoxicity.
Endosomal sorting related protein CHMP2B is localized in Lewy bodies and glial cytoplasmic inclusions in α-synucleinopathy.
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects.
Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP.
Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.
Convergent transcription induces transcriptional gene silencing in fission yeast and mammalian cells.
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs.
Molecular analysis and biochemical classification of TDP-43 proteinopathy.
Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study.
The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.
Long non-coding RNA gadd7 interacts with TDP-43 and regulates Cdk6 mRNA decay.
An early onset progressive motor neuron disorder in Scyl1-deficient mice is associated with mislocalization of TDP-43.
Minocycline-associated rimmed vacuolar myopathy in a patient with rheumatoid arthritis.
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity.
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.
Wild type TDP-43 induces neuro-inflammation and alters APP metabolism in lentiviral gene transfer models.
First neuropathological description of a patient with Parkinson's disease and LRRK2 p.N1437H mutation.
Essential tremor with ubiquitinated intranuclear inclusions and cerebellar degeneration.
TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.
Quantitative analysis of the detergent-insoluble brain proteome in frontotemporal lobar degeneration using SILAC internal standards.
Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy.
Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates.
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR.
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.
Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.
Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p.
Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations.
Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p.
Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span.
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking.
Phosphorylated tau/amyloid beta 1-42 ratio in ventricular cerebrospinal fluid reflects outcome in idiopathic normal pressure hydrocephalus.
Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region.
The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons.
Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan.
Abnormal tau deposition in neurons, but not in glial cells in the cerebral tissue surrounding arteriovenous malformation.
Neuropathology in classical and variant ataxia-telangiectasia.
Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43).
Rodent models of TDP-43: recent advances.
Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation.
Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices.
HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice.
TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans.
Prevalence, laterality, and comorbidity of hippocampal sclerosis in an autopsy sample.
ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications.
Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan).
A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy.
Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy.
Transglutaminase 1 and its regulator tazarotene-induced gene 3 localize to neuronal tau inclusions in tauopathies.
Right temporal variant frontotemporal dementia with motor neuron disease.
Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43.
Supranuclear ophthalmoparesis and vacuolar degeneration of the cerebral white matter in amyotrophic lateral sclerosis: a clinicopathological study.
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach.
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.
Epitope mapping of antibodies against TDP-43 and detection of protease-resistant fragments of pathological TDP-43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Alteration in cell cycle-related proteins in lymphoblasts from carriers of the c.709-1G>A PGRN mutation associated with FTLD-TDP dementia.
Unusual clinical presentation and neuropathology in two subjects with fused-in sarcoma (FUS) positive inclusions.
TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes.
Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS.
Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker.
Genetic association of CR1 with Alzheimer's disease: a tentative disease mechanism.
Ultrastructure of ubiquitin-positive, TDP-43-negative neuronal inclusions in cerebral cortex of C9ORF72-linked frontotemporal lobar degeneration/amyotrophic lateral sclerosis.
Familial ALS with FUS P525L mutation: two Japanese sisters with multiple systems involvement.
Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis.
Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.
Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.
Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch.
Reduced expression of BTBD10, an Akt activator, leads to motor neuron death.
TDP-43 pathology in a case of hereditary spastic paraplegia with a NIPA1/SPG6 mutation.
Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions.
Asparaginyl endopeptidase cleaves TDP-43 in brain.
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis.
TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease.
Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice.
Drug screening for ALS using patient-specific induced pluripotent stem cells.
Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.
Aquaporin-4 expression in distal myopathy with rimmed vacuoles.
Comparison of phosphorylated TDP-43-positive inclusions in oculomotor neurons in patients with non-ALS and ALS disorders.
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.
Prevention of motor neuron degeneration by novel iron chelators in SOD1(G93A) transgenic mice of amyotrophic lateral sclerosis.
Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons.
Proteomic analysis of hippocampal dentate granule cells in frontotemporal lobar degeneration: application of laser capture technology.
A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis.
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis.
Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.
The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships.
Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology.
Proportional neuronal loss between the primary motor and sensory cortex in amyotrophic lateral sclerosis.
Immunohistochemical study of microscopic globular bodies of normal human brain.
An autopsy case of an aged patient with spinocerebellar ataxia type 2.
Diagnostic value of markers of muscle degeneration in sporadic inclusion body myositis.
Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo.
TDP-43 variants of frontotemporal lobar degeneration.
Long-term observational study of sporadic inclusion body myositis.
An autopsy case of SOD1-related ALS with TDP-43 positive inclusions.
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways.
Hippocampal sclerosis in advanced age: clinical and pathological features.
Granular expression of prolyl-peptidyl isomerase PIN1 is a constant and specific feature of Alzheimer's disease pathology and is independent of tau, Aβ and TDP-43 pathology.
Fronto-temporal lobar degeneration: neuropathology in 60 cases.
TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling.
A family with Parkinsonism, essential tremor, restless legs syndrome, and depression.
FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases.
A novel double mutation in FUS gene causing sporadic ALS.
TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor.
New neuropathological findings in Unverricht-Lundborg disease: neuronal intranuclear and cytoplasmic inclusions.
Pathological correlates of frontotemporal lobar degeneration in the elderly.
Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: specificity, sensitivity, and a possible prognostic value.
TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice.
TDP-43 pathology occurs infrequently in multiple system atrophy.
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie).
Ubiquitinated, p62 immunopositive cerebellar cortical neuronal inclusions are evident across the spectrum of TDP-43 proteinopathies but are only rarely additionally immunopositive for phosphorylation-dependent TDP-43.
Catastrophic hyperkalaemia following administration of suxamethonium chloride to a patient with undiagnosed amyotrophic lateral sclerosis.
An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity.
RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS.
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene.
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS.
Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1.
TDP-43 regulates its mRNA levels through a negative feedback loop.
FALS with Gly72Ser mutation in SOD1 gene: report of a family including the first autopsy case.
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes.
Biochemical, neuropathological, and neuroimaging characteristics of early-onset Alzheimer's disease due to a novel PSEN1 mutation.
Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family.
Overview and recent advances in neuropathology. Part 2: Neurodegeneration.
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies.
A morphometric study of the spatial patterns of TDP-43 immunoreactive neuronal inclusions in frontotemporal lobar degeneration (FTLD) with progranulin (GRN) mutation.
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation.
Inorganic mercury within motor neurons does not cause the TDP-43 changes seen in sporadic ALS.
Clinicopathological study of diffuse neurofibrillary tangles with calcification. With special reference to TDP-43 proteinopathy and alpha-synucleinopathy.
The ALS/PDC syndrome of Guam: potential biomarkers for an enigmatic disorder.
Abnormal TDP-43 and FUS proteins in muscles of sporadic IBM: similarities in a TARDBP-linked ALS patient.
Molecular properties of TAR DNA binding protein-43 fragments are dependent upon its cleavage site.
Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans.
TDP-43 pathological changes in early onset familial and sporadic Alzheimer's disease, late onset Alzheimer's disease and Down's syndrome: association with age, hippocampal sclerosis and clinical phenotype.
Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model.
FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis.
Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations.
Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Enhancement of native and phosphorylated TDP-43 immunoreactivity by proteinase K treatment following autoclave heating.
Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation.
A peculiar constellation of tau pathology defines a subset of dementia in the elderly.
C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress.
TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6.
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.
Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
In sporadic inclusion body myositis muscle fibres TDP-43-positive inclusions are less frequent and robust than p62 inclusions, and are not associated with paired helical filaments.
Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosome 9p.
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.
Novel histopathologic findings in molecularly-confirmed pantothenate kinase-associated neurodegeneration.
TDP-43 pathology may occur in the BRI2 gene-related dementias.
Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy.
Novel progranulin mutation detected in 2 patients with FTLD.
ApoE E4 is a susceptibility factor in amnestic but not aphasic dementias.
β-amyloid triggers ALS-associated TDP-43 pathology in AD models.
TDP-43-induced death is associated with altered regulation of BIM and Bcl-xL and attenuated by caspase-mediated TDP-43 cleavage.
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1.
TDP43-positive intraneuronal inclusions in a patient with motor neuron disease and Parkinson's disease.
TDP-43 M337V mutation in familial amyotrophic lateral sclerosis in Japan.
Intra-familial clinical heterogeneity due to FTLD-U with TDP-43 proteinopathy caused by a novel deletion in progranulin gene (PGRN).
Increased neuronal Rab5 immunoreactive endosomes do not colocalize with TDP-43 in motor neuron disease.
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.
Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism.
Lack of shunt response in suspected idiopathic normal pressure hydrocephalus with Alzheimer disease pathology.
Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness.
Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice.
Involvement of Onuf's nucleus in Machado-Joseph disease: a morphometric and immunohistochemical study.
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.
Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy.
Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue.
Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease.
Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions.
Increased caspase activation and decreased TDP-43 solubility in progranulin knockout cortical cultures.
Age-dependent changes in TDP-43 levels in a mouse model of Alzheimer disease are linked to Aβ oligomers accumulation.
Efficient conditional and promoter-specific in vivo expression of cDNAs of choice by taking advantage of recombinase-mediated cassette exchange using FlEx gene traps.
TDP-43 dimerizes in human cells in culture.
FUS-immunoreactive intranuclear inclusions in neurodegenerative disease.
A mutation affecting the sodium/proton exchanger, SLC9A6, causes mental retardation with tau deposition.
TDP-43 and ubiquitinated cytoplasmic aggregates in sporadic ALS are low frequency and widely distributed in the lower motor neuron columns independent of disease spread.
Mutant superoxide dismutase 1 overexpression in NSC-34 cells: effect of trehalose on aggregation, TDP-43 localization and levels of co-expressed glycoproteins.
Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology.
Mutations of optineurin in amyotrophic lateral sclerosis.
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Synergistic effect between proteasome and autophagosome in the clearance of polyubiquitinated TDP-43.
TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.
Sporadic corticobasal syndrome due to FTLD-TDP.
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking.
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis.
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis.
Relationship between Bunina bodies and TDP-43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis.
International Workshop on Inclusion Body Myositis held at the Institute of Myology, Paris, on 29 May 2009.
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration.
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
Neuropathology of olfactory ensheathing cell transplantation into the brain of two amyotrophic lateral sclerosis (ALS) patients.
Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging.
Caudate atrophy on MRI is a characteristic feature of FTLD-FUS.
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2.
TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease.
Alterations in subcellular localization of TDP-43 immunoreactivity in the anterior horns in sporadic amyotrophic lateral sclerosis.
Sustained expression of TDP-43 and FUS in motor neurons in rodent's lifetime.
A novel X-linked four-repeat tauopathy with Parkinsonism and spasticity.
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.
Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease.
Modeling the association between 43 different clinical and pathological variables and the severity of cognitive impairment in a large autopsy cohort of elderly persons.
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases.
Brain progranulin expression in GRN-associated frontotemporal lobar degeneration.
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6.
In vivo amyloid imaging in autopsy-confirmed Parkinson disease with dementia.
Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice.
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.
Alzheimer disease-like phenotype associated with the c.154delA mutation in progranulin.
Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis.
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.
TDP-43 is a developmentally regulated protein essential for early embryonic development.
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration.
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo.
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease.
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase.
TDP-43 proteinopathy in familial motor neurone disease with TARDBP A315T mutation: a case report.
Widespread occurrence of eosinophilic neuronal cytoplasmic inclusions in an asymptomatic adult: a novel ubiquitin-negative filamentous inclusion.
Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology.
Exome sequencing reveals VCP mutations as a cause of familial ALS.
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism.
Phosphoproteomic analysis reveals site-specific changes in GFAP and NDRG2 phosphorylation in frontotemporal lobar degeneration.
Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures.
Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration?
TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia.
Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis.
Abnormal TDP-43 expression is identified in the neocortex in cases of dementia pugilistica, but is mainly confined to the limbic system when identified in high and moderate stages of Alzheimer's disease.
Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.
TDP-43-mediated neuron loss in vivo requires RNA-binding activity.
Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations.
Neurotoxic effects of TDP-43 overexpression in C. elegans.
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chains.
Survival in progressive supranuclear palsy and frontotemporal dementia.
Olfactory epithelium amyloid-beta and paired helical filament-tau pathology in Alzheimer disease.
FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion.
Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study.
Alteration of biochemical and pathological properties of TDP-43 protein by a lipid mediator, 15-deoxy-Delta(12,14)-prostaglandin J(2).
Alzheimer's disease phenotypes and genotypes associated with mutations in presenilin 2.
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis.
Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS).
Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase.
Neuropathological investigation of regions responsible for semantic aphasia in frontotemporal lobar degeneration.
Abnormal localization of leucine-rich repeat kinase 2 to the endosomal-lysosomal compartment in lewy body disease.
TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons.
TDP-43 in ubiquitinated inclusions in the inferior olives in frontotemporal lobar degeneration and in other neurodegenerative diseases: a degenerative process distinct from normal ageing.
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes.
Chemical manipulation of hsp70 ATPase activity regulates tau stability.
Phosphorylation-dependent TDP-43 antibody detects intraneuronal dot-like structures showing morphological characters of granulovacuolar degeneration.
Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching.
TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea.
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
Regional distribution of TDP-43 inclusions in Alzheimer disease (AD) brains: their relation to AD common pathology.
Accumulation of TDP-43 and alpha-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation.
Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado-Joseph disease.
Progranulin is expressed within motor neurons and promotes neuronal cell survival.
Decreased cystatin C immunoreactivity in spinal motor neurons and astrocytes in amyotrophic lateral sclerosis.
Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease.
A new subtype of frontotemporal lobar degeneration with FUS pathology.
Plasma phosphorylated-TDP-43 protein levels correlate with brain pathology in frontotemporal lobar degeneration.
TARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy.
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease.
TDP-43 is recruited to stress granules in conditions of oxidative insult.
Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury.
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
Prominent phenotypic variability associated with mutations in Progranulin.
Pallidonigral TDP-43 pathology in Perry syndrome.
VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death.
TDP-43 is not present in brain tissue of patients with schizophrenia.
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.
Inflammatory myopathies with mitochondrial pathology and protein aggregates.
Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy.
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.
Deep brain stimulation in dystonia: sonographic monitoring of electrode placement into the globus pallidus internus.
No Lewy pathology in monkeys with over 10 years of severe MPTP Parkinsonism.
Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.
Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS.
Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis.
Abstracts of the 110th Meeting of the British Neuropathological Society. January 7-9, 2009. London, United Kingdom.
Neuropathology and applied neurobiology 2009 Jan;35 Suppl 1:1-38
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology.
Progression of hippocampal degeneration in amyotrophic lateral sclerosis with or without memory impairment: distinction from Alzheimer disease.
Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein.
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury.
Clinical and pathological characteristics of patients with leucine-rich repeat kinase-2 mutations.
Rapidly progressive neurodegenerative dementias.
Clinical and pathological continuum of multisystem TDP-43 proteinopathies.
TDP-43 accumulation is common in myopathies with rimmed vacuoles.
Accumulation of phosphorylated TDP-43 in brains of patients with argyrophilic grain disease.
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies.
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6.
Absence of Pittsburgh compound B detection of cerebral amyloid beta in a patient with clinical, cognitive, and cerebrospinal fluid markers of Alzheimer disease: a case report.
Transactive response DNA-binding protein 43 burden in familial Alzheimer disease and Down syndrome.
Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP-43 pathology in a patient after long-term survival on a respirator.
TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations.
Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons.
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).
The dorsal raphe nucleus shows phospho-tau neurofibrillary changes before the transentorhinal region in Alzheimer's disease. A precocious onset?
Frontotemporal lobar degeneration with ubiquitinated tau-negative inclusions and additional alpha-synuclein pathology but also unusual cerebellar ubiquitinated p62-positive, TDP-43-negative inclusions.
TDP-43 pathology in familial British dementia.
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin.
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases.
TDP-43 neuropathology is similar in sporadic amyotrophic lateral sclerosis with or without TDP-43 mutations.
Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration.
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.
Neuropathologic classification of dementias: introduction.
Frontotemporal dementia mimicking dementia with Lewy bodies.
Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease.
Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis.
TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia.
White matter tauopathy with globular glial inclusions: a distinct sporadic frontotemporal lobar degeneration.
Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease.
TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Progranulin mutation causes frontotemporal dementia in the Swedish Karolinska family.
Structural determinants of the cellular localization and shuttling of TDP-43.
Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis.
Pilot study of granulocyte colony stimulating factor (G-CSF)-mobilized peripheral blood stem cells in amyotrophic lateral sclerosis (ALS).
Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions.
Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study.
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation.
Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype.
Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations.
Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model.
TDP-43 immunoreactivity in anoxic, ischemic and neoplastic lesions of the central nervous system.
Epitope mapping of 2E2-D3, a monoclonal antibody directed against human TDP-43.
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis.
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies.
Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia.
A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro.
Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS.
Progranulin gene mutation with an unusual clinical and neuropathologic presentation.
Update on recent molecular and genetic advances in frontotemporal lobar degeneration.
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Clinical, neuropathological and genotypic variability in SNCA A53T familial Parkinson's disease. Variability in familial Parkinson's disease.
Early onset familial Alzheimer Disease with spastic paraparesis, dysarthria, and seizures and N135S mutation in PSEN1.
TAR-DNA binding protein 43 in Pick disease.
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam.
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease.
TDP-43-immunoreactive neuronal and glial inclusions in the neostriatum in amyotrophic lateral sclerosis with and without dementia.
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.
Liver X receptor beta (LXRbeta): a link between beta-sitosterol and amyotrophic lateral sclerosis-Parkinson's dementia.
Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease.
LRRK2 and parkin immunoreactivity in multiple system atrophy inclusions.
Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC).
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy.
Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia.
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases.
TDP-43 inclusions do not protect motor neurons from sporadic ALS.
Temporal lobar predominance of TDP-43 neuronal cytoplasmic inclusions in Alzheimer disease.
Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases.
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia.
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions.
Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions.
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD.
TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer's disease and frontotemporal lobar degeneration.
Pedigree with frontotemporal lobar degeneration--motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9.
Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions visualized with ubiquitin-binding protein p62 immunohistochemistry.
TAR DNA-binding protein 43 immunohistochemistry reveals extensive neuritic pathology in FTLD-U: a midwest-southwest consortium for FTLD study.
TDP-43 mutation in familial amyotrophic lateral sclerosis.
Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases.
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43.
Corticobasal syndrome associated with the A9D Progranulin mutation.
A reassessment of the neuropathology of frontotemporal dementia linked to chromosome 3.
Alzheimer and Parkinson diagnoses in progranulin null mutation carriers in an extended founder family.
Progressive anomia revisited: focal degeneration associated with progranulin gene mutation.
Frontotemporal lobar degeneration with upper motor neuron disease/ primary lateral sclerosis.
Increase in the relative expression of tau with four microtubule binding repeat regions in frontotemporal lobar degeneration and progressive supranuclear palsy brains.
Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease.
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease.
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.
TDP-43 is deposited in the Guam parkinsonism-dementia complex brains.
A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology.
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.
Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43.
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Clinicopathologic features of frontotemporal dementia with progranulin sequence variation.
Nuclear protein profiling of Jurkat cells during heat stress-induced apoptosis by 2-DE and MS/MS.
Appearance pattern of TDP-43 in Japanese frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions.
TDP-43 in differential diagnosis of motor neuron disorders.
A 34-year-old man with progressive behavioral and language disturbance.
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations.
Evidence that TDP-43 is not the major ubiquitinated target within the pathological inclusions of amyotrophic lateral sclerosis.
Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies.
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Thomas JL, Nilaver BI, Lomniczi A, Brown DI, Appleman ML, Kohama SG, Urbanski HF
Journal of Alzheimer's disease reports 2024;8(1):25-32
Journal of Alzheimer's disease reports 2024;8(1):25-32
Evaluation of cyanotoxin L-BMAA effect on α-synuclein and TDP43 proteinopathy.
Sini P, Galleri G, Ciampelli C, Galioto M, Padedda BM, Lugliè A, Iaccarino C, Crosio C
Frontiers in immunology 2024;15:1360068
Frontiers in immunology 2024;15:1360068
Biochemical, Biomarker, and Behavioral Characterization of the Grn(R493X) Mouse Model of Frontotemporal Dementia.
Smith DM, Aggarwal G, Niehoff ML, Jones SA, Banerjee S, Farr SA, Nguyen AD
Molecular neurobiology 2024 Nov;61(11):9708-9722
Molecular neurobiology 2024 Nov;61(11):9708-9722
Pathologic TDP-43 downregulates myelin gene expression in the monkey brain.
Zhu L, Bai D, Wang X, Ou K, Li B, Jia Q, Tan Z, Liang J, He D, Yan S, Wang L, Li S, Li XJ, Yin P
Brain pathology (Zurich, Switzerland) 2024 Nov;34(6):e13277
Brain pathology (Zurich, Switzerland) 2024 Nov;34(6):e13277
Involvement of CB(1) and CB(2) receptors in neuroprotective effects of cannabinoids in experimental TDP-43 related frontotemporal dementia using male mice.
Gonzalo-Consuegra C, Santos-García I, García-Toscano L, Martín-Baquero R, Rodríguez-Cueto C, Wittwer MB, Dzygiel P, Grether U, de Lago E, Fernández-Ruiz J
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie 2024 May;174:116473
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie 2024 May;174:116473
A TBK1 variant causes autophagolysosomal and motoneuron pathology without neuroinflammation in mice.
Brenner D, Sieverding K, Srinidhi J, Zellner S, Secker C, Yilmaz R, Dyckow J, Amr S, Ponomarenko A, Tunaboylu E, Douahem Y, Schlag JS, Rodríguez Martínez L, Kislinger G, Niemann C, Nalbach K, Ruf WP, Uhl J, Hollenbeck J, Schirmer L, Catanese A, Lobsiger CS, Danzer KM, Yilmazer-Hanke D, Münch C, Koch P, Freischmidt A, Fetting M, Behrends C, Parlato R, Weishaupt JH
The Journal of experimental medicine 2024 May 6;221(5)
The Journal of experimental medicine 2024 May 6;221(5)
The new missense G376V-TDP-43 variant induces late-onset distal myopathy but not amyotrophic lateral sclerosis.
Zibold J, Lessard LER, Picard F, da Silva LG, Zadorozhna Y, Streichenberger N, Belotti E, Osseni A, Emerit A, Errazuriz-Cerda E, Michel-Calemard L, Menassa R, Coudert L, Wiessner M, Stucka R, Klopstock T, Simonetti F, Hutten S, Nonaka T, Hasegawa M, Strom TM, Bernard E, Ollagnon E, Urtizberea A, Dormann D, Petiot P, Schaeffer L, Senderek J, Leblanc P
Brain : a journal of neurology 2024 May 3;147(5):1768-1783
Brain : a journal of neurology 2024 May 3;147(5):1768-1783
Serum GFAP levels correlate with astrocyte reactivity, post-mortem brain atrophy and neurofibrillary tangles.
Sánchez-Juan P, Valeriano-Lorenzo E, Ruiz-González A, Pastor AB, Rodrigo Lara H, López-González F, Zea-Sevilla MA, Valentí M, Frades B, Ruiz P, Saiz L, Burgueño-García I, Calero M, Del Ser T, Rábano A
Brain : a journal of neurology 2024 May 3;147(5):1667-1679
Brain : a journal of neurology 2024 May 3;147(5):1667-1679
RNA-binding properties orchestrate TDP-43 homeostasis through condensate formation in vivo.
Scherer NM, Maurel C, Graus MS, McAlary L, Richter G, Radford RAW, Hogan A, Don EK, Lee A, Yerbury J, Francois M, Chung RS, Morsch M
Nucleic acids research 2024 May 22;52(9):5301-5319
Nucleic acids research 2024 May 22;52(9):5301-5319
Time-resolved profiling of RNA binding proteins throughout the mRNA life cycle.
Choi Y, Um B, Na Y, Kim J, Kim JS, Kim VN
Molecular cell 2024 May 2;84(9):1764-1782.e10
Molecular cell 2024 May 2;84(9):1764-1782.e10
CAG repeat mosaicism is gene specific in spinocerebellar ataxias.
Kacher R, Lejeune FX, David I, Boluda S, Coarelli G, Leclere-Turbant S, Heinzmann A, Marelli C, Charles P, Goizet C, Kabir N, Hilab R, Jornea L, Six J, Dommergues M, Fauret AL, Brice A, Humbert S, Durr A
American journal of human genetics 2024 May 2;111(5):913-926
American journal of human genetics 2024 May 2;111(5):913-926
DBT is a metabolic switch for maintenance of proteostasis under proteasomal impairment.
Hwang RD, Lu Y, Tang Q, Periz G, Park G, Li X, Xiang Q, Liu Y, Zhang T, Wang J
bioRxiv : the preprint server for biology 2024 May 2;
bioRxiv : the preprint server for biology 2024 May 2;
Oxidative cyclization reagents reveal tryptophan cation-π interactions.
Xie X, Moon PJ, Crossley SWM, Bischoff AJ, He D, Li G, Dao N, Gonzalez-Valero A, Reeves AG, McKenna JM, Elledge SK, Wells JA, Toste FD, Chang CJ
Nature 2024 Mar;627(8004):680-687
Nature 2024 Mar;627(8004):680-687
Colocalization of TDP-43 and stress granules at the early stage of TDP-43 aggregation in amyotrophic lateral sclerosis.
Mori F, Yasui H, Miki Y, Kon T, Arai A, Kurotaki H, Tomiyama M, Wakabayashi K
Brain pathology (Zurich, Switzerland) 2024 Mar;34(2):e13215
Brain pathology (Zurich, Switzerland) 2024 Mar;34(2):e13215
Casein kinase 1 inhibitor avoids TDP-43 pathology propagation in a patient-derived cellular model of amyotrophic lateral sclerosis.
Cuevas EP, Martinez-Gonzalez L, Gordillo C, Tosat-Bitrián C, Pérez de la Lastra C, Sáenz A, Gil C, Palomo V, Martin-Requero Á, Martinez A
Neurobiology of disease 2024 Mar;192:106430
Neurobiology of disease 2024 Mar;192:106430
Large-scale RNA-seq mining reveals ciclopirox triggers TDP-43 cryptic exons.
Sinha IR, Sandal PS, Burns GD, Mallika AP, Irwin KE, Cruz ALF, Wang V, Rodríguez JL, Wong PC, Ling JP
bioRxiv : the preprint server for biology 2024 Mar 30;
bioRxiv : the preprint server for biology 2024 Mar 30;
The nuclear import receptor Kapβ2 modifies neurotoxicity mediated by poly(GR) in C9orf72-linked ALS/FTD.
Cicardi ME, Kankate V, Sriramoji S, Krishnamurthy K, Markandaiah SS, Verdone BM, Girdhar A, Nelson A, Rivas LB, Boehringer A, Haeusler AR, Pasinelli P, Guo L, Trotti D
Communications biology 2024 Mar 28;7(1):376
Communications biology 2024 Mar 28;7(1):376
Endogenous TDP-43 mislocalization in a novel knock-in mouse model reveals DNA repair impairment, inflammation, and neuronal senescence.
Mitra J, Dharmalingam P, Kodavati M, Guerrero EN, Rao KS, Garruto RM, Hegde ML
Research square 2024 Mar 20;
Research square 2024 Mar 20;
Reduced STMN2 and pathogenic TDP-43, two hallmarks of ALS, synergize to accelerate motor decline in mice.
Krus KL, Benitez AM, Strickland A, Milbrandt J, Bloom AJ, DiAntonio A
bioRxiv : the preprint server for biology 2024 Mar 20;
bioRxiv : the preprint server for biology 2024 Mar 20;
Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons.
Lépine S, Nauleau-Javaudin A, Deneault E, Chen CX, Abdian N, Franco-Flores AK, Haghi G, Castellanos-Montiel MJ, Maussion G, Chaineau M, Durcan TM
iScience 2024 Mar 15;27(3):109166
iScience 2024 Mar 15;27(3):109166
Regulating PCCA gene expression by modulation of pseudoexon splicing patterns to rescue enzyme activity in propionic acidemia.
Spangsberg Petersen US, Dembic M, Martínez-Pizarro A, Richard E, Holm LL, Havelund JF, Doktor TK, Larsen MR, Færgeman NJ, Desviat LR, Andresen BS
Molecular therapy. Nucleic acids 2024 Mar 12;35(1):102101
Molecular therapy. Nucleic acids 2024 Mar 12;35(1):102101
Computational prediction and experimental validation identify functionally conserved lncRNAs from zebrafish to human.
Huang W, Xiong T, Zhao Y, Heng J, Han G, Wang P, Zhao Z, Shi M, Li J, Wang J, Wu Y, Liu F, Xi JJ, Wang Y, Zhang QC
Nature genetics 2024 Jan;56(1):124-135
Nature genetics 2024 Jan;56(1):124-135
Loss of TDP-43 mediates severe neurotoxicity by suppressing PJA1 gene transcription in the monkey brain.
Zhu L, Deng F, Bai D, Hou J, Jia Q, Zhang C, Ou K, Li S, Li XJ, Yin P
Cellular and molecular life sciences : CMLS 2024 Jan 9;81(1):16
Cellular and molecular life sciences : CMLS 2024 Jan 9;81(1):16
Cryptic splicing of stathmin-2 and UNC13A mRNAs is a pathological hallmark of TDP-43-associated Alzheimer's disease.
Agra Almeida Quadros AR, Li Z, Wang X, Ndayambaje IS, Aryal S, Ramesh N, Nolan M, Jayakumar R, Han Y, Stillman H, Aguilar C, Wheeler HJ, Connors T, Lopez-Erauskin J, Baughn MW, Melamed Z, Beccari MS, Olmedo Martínez L, Canori M, Lee CZ, Moran L, Draper I, Kopin AS, Oakley DH, Dickson DW, Cleveland DW, Hyman BT, Das S, Ertekin-Taner N, Lagier-Tourenne C
Acta neuropathologica 2024 Jan 4;147(1):9
Acta neuropathologica 2024 Jan 4;147(1):9
Cross-seeding by prion protein inactivates TDP-43.
Polido SA, Stuani C, Voigt A, Banik P, Kamps J, Bader V, Grover P, Krause LJ, Zerr I, Matschke J, Glatzel M, Winklhofer KF, Buratti E, Tatzelt J
Brain : a journal of neurology 2024 Jan 4;147(1):240-254
Brain : a journal of neurology 2024 Jan 4;147(1):240-254
TDP43/HDAC6/Prdx1 signaling pathway participated in the cognitive impairment of obstructive sleep apnea via regulating inflammation and oxidative stress.
Ou Y, Shen C, Chen Z, Liu T, Peng Y, Zong D, Ouyang R
International immunopharmacology 2024 Jan 25;127:111350
International immunopharmacology 2024 Jan 25;127:111350
TDP-43 nuclear loss in FTD/ALS causes widespread alternative polyadenylation changes.
Zeng Y, Lovchykova A, Akiyama T, Liu C, Guo C, Jawahar VM, Sianto O, Calliari A, Prudencio M, Dickson DW, Petrucelli L, Gitler AD
bioRxiv : the preprint server for biology 2024 Jan 22;
bioRxiv : the preprint server for biology 2024 Jan 22;
TDP-43 impairs sleep in Drosophila through Ataxin-2-dependent metabolic disturbance.
Perlegos AE, Durkin J, Belfer SJ, Rodriguez A, Shcherbakova O, Park K, Luong J, Bonini NM, Kayser MS
Science advances 2024 Jan 12;10(2):eadj4457
Science advances 2024 Jan 12;10(2):eadj4457
TDP-43 pathology links innate and adaptive immunity in amyotrophic lateral sclerosis.
Evangelista BA, Ragusa JV, Pellegrino K, Wu Y, Quiroga-Barber IY, Cahalan SR, Arooji OK, Madren JA, Schroeter S, Cozzarin J, Xie L, Chen X, White KK, Ezzell JA, Iannone MA, Cohen S, Traub RE, Li X, Bedlack R, Phanstiel DH, Meeker R, Stanley N, Cohen TJ
bioRxiv : the preprint server for biology 2024 Jan 10;
bioRxiv : the preprint server for biology 2024 Jan 10;
IκB kinase phosphorylates cytoplasmic TDP-43 and promotes its proteasome degradation.
Iguchi Y, Takahashi Y, Li J, Araki K, Amakusa Y, Kawakami Y, Kobayashi K, Yokoi S, Katsuno M
The Journal of cell biology 2024 Feb 5;223(2)
The Journal of cell biology 2024 Feb 5;223(2)
Mouse oocytes sequester aggregated proteins in degradative super-organelles.
Zaffagnini G, Cheng S, Salzer MC, Pernaute B, Duran JM, Irimia M, Schuh M, Böke E
Cell 2024 Feb 29;187(5):1109-1126.e21
Cell 2024 Feb 29;187(5):1109-1126.e21
hnRNPA2B1 represses the disassembly of arsenite-induced stress granules and is essential for male fertility.
Wang X, Fan X, Zhang J, Wang F, Chen J, Wen Y, Wang L, Li T, Li H, Gu H, Zhang Y, Yuan S
Cell reports 2024 Feb 27;43(2):113769
Cell reports 2024 Feb 27;43(2):113769
Age-Related Pathology in Corticobasal Degeneration.
Mimuro M, Iwasaki Y
International journal of molecular sciences 2024 Feb 27;25(5)
International journal of molecular sciences 2024 Feb 27;25(5)
Restoring functional TDP-43 oligomers in ALS and laminopathic cellular models through baicalein-induced reconfiguration of TDP-43 aggregates.
Chang HY, Wang IF
Scientific reports 2024 Feb 26;14(1):4620
Scientific reports 2024 Feb 26;14(1):4620
Biochemical, biomarker, and behavioral characterization of the Grn(R493X) mouse model of frontotemporal dementia.
Smith DM, Aggarwal G, Niehoff ML, Jones SA, Banerjee S, Farr SA, Nguyen AD
bioRxiv : the preprint server for biology 2024 Feb 2;
bioRxiv : the preprint server for biology 2024 Feb 2;
Regulation of stress granule formation in human oligodendrocytes.
Pernin F, Cui QL, Mohammadnia A, Fernandes MGF, Hall JA, Srour M, Dudley RWR, Zandee SEJ, Klement W, Prat A, Salapa HE, Levin MC, Moore GRW, Kennedy TE, Vande Velde C, Antel JP
Nature communications 2024 Feb 19;15(1):1524
Nature communications 2024 Feb 19;15(1):1524
Reduced progranulin increases tau and α-synuclein inclusions and alters mouse tauopathy phenotypes via glucocerebrosidase.
Takahashi H, Bhagwagar S, Nies SH, Ye H, Han X, Chiasseu MT, Wang G, Mackenzie IR, Strittmatter SM
Nature communications 2024 Feb 16;15(1):1434
Nature communications 2024 Feb 16;15(1):1434
Progranulin haploinsufficiency mediates cytoplasmic TDP-43 aggregation with lysosomal abnormalities in human microglia.
Sung W, Noh MY, Nahm M, Kim YS, Ki CS, Kim YE, Kim HJ, Kim SH
Journal of neuroinflammation 2024 Feb 13;21(1):47
Journal of neuroinflammation 2024 Feb 13;21(1):47
A Panel of miRNA Biomarkers Common to Serum and Brain-Derived Extracellular Vesicles Identified in Mouse Model of Amyotrophic Lateral Sclerosis.
Vassileff N, Spiers JG, Lee JD, Woodruff TM, Ebrahimie E, Mohammadi Dehcheshmeh M, Hill AF, Cheng L
Molecular neurobiology 2024 Aug;61(8):5901-5915
Molecular neurobiology 2024 Aug;61(8):5901-5915
Retrospective neuropathological diagnosis of TDP-43 proteinopathies: Factors affecting immunoreactivity of phosphorylated TDP-43 in fixed post-mortem brain tissue.
Robinson AC, Davidson YS, Minshull J, Lally I, Walker L, Mann DMA, Roncaroli F
Neuropathology : official journal of the Japanese Society of Neuropathology 2024 Apr;44(2):173-179
Neuropathology : official journal of the Japanese Society of Neuropathology 2024 Apr;44(2):173-179
Exploring Options for Proximity-Dependent Biotinylation Experiments: Comparative Analysis of Labeling Enzymes and Affinity Purification Resins.
Schreiber KJ, Kadijk E, Youn JY
Journal of proteome research 2024 Apr 5;23(4):1531-1543
Journal of proteome research 2024 Apr 5;23(4):1531-1543
KCNJ2 inhibition mitigates mechanical injury in a human brain organoid model of traumatic brain injury.
Lai JD, Berlind JE, Fricklas G, Lie C, Urenda JP, Lam K, Sta Maria N, Jacobs R, Yu V, Zhao Z, Ichida JK
Cell stem cell 2024 Apr 4;31(4):519-536.e8
Cell stem cell 2024 Apr 4;31(4):519-536.e8
Seeding competent TDP-43 persists in human patient and mouse muscle.
Lynch EM, Pittman S, Daw J, Ikenaga C, Chen S, Dhavale DD, Jackrel ME, Ayala YM, Kotzbauer P, Ly CV, Pestronk A, Lloyd TE, Weihl CC
bioRxiv : the preprint server for biology 2024 Apr 4;
bioRxiv : the preprint server for biology 2024 Apr 4;
Design principles to tailor Hsp104 therapeutics.
Lin J, Carman PJ, Gambogi CW, Kendsersky NM, Chuang E, Gates SN, Yokom AL, Rizo AN, Southworth DR, Shorter J
bioRxiv : the preprint server for biology 2024 Apr 28;
bioRxiv : the preprint server for biology 2024 Apr 28;
Depletion of Mettl3 in cholinergic neurons causes adult-onset neuromuscular degeneration.
Dermentzaki G, Furlan M, Tanaka I, Leonardi T, Rinchetti P, Passos PMS, Bastos A, Ayala YM, Hanna JH, Przedborski S, Bonanomi D, Pelizzola M, Lotti F
Cell reports 2024 Apr 23;43(4):113999
Cell reports 2024 Apr 23;43(4):113999
Reversal of C9orf72 mutation-induced transcriptional dysregulation and pathology in cultured human neurons by allele-specific excision.
Sachdev A, Gill K, Sckaff M, Birk AM, Aladesuyi Arogundade O, Brown KA, Chouhan RS, Issagholian-Lewin PO, Patel E, Watry HL, Bernardi MT, Keough KC, Tsai YC, Smith AST, Conklin BR, Clelland CD
Proceedings of the National Academy of Sciences of the United States of America 2024 Apr 23;121(17):e2307814121
Proceedings of the National Academy of Sciences of the United States of America 2024 Apr 23;121(17):e2307814121
Anterior insula is more vulnerable than posterior insula to TDP-43 pathology in common dementias and ALS.
Lochner RH, Arumanayagam AS, Powell SZ, Masdeu JC, Pascual B, Cykowski MD
Journal of neuropathology and experimental neurology 2024 Apr 19;83(5):307-317
Journal of neuropathology and experimental neurology 2024 Apr 19;83(5):307-317
TDP-43 deficiency in suprachiasmatic nucleus perturbs rhythmicity of neuroactivity in prefrontal cortex.
Zhang H, Chen C, Zhang EE, Huang X
iScience 2024 Apr 19;27(4):109522
iScience 2024 Apr 19;27(4):109522
Targeting 14-3-3θ-mediated TDP-43 pathology in amyotrophic lateral sclerosis and frontotemporal dementia mice.
Ke YD, van Hummel A, Au C, Chan G, Lee WS, van der Hoven J, Przybyla M, Deng Y, Sabale M, Morey N, Bertz J, Feiten A, Ippati S, Stevens CH, Yang S, Gladbach A, Haass NK, Kril JJ, Blair IP, Delerue F, Ittner LM
Neuron 2024 Apr 17;112(8):1249-1264.e8
Neuron 2024 Apr 17;112(8):1249-1264.e8
VCP-related myopathy: a case series and a review of literature.
Iannibelli E, Gibertini S, Cheli M, Blasevich F, Cavaliere A, Riolo G, Ruggieri A, Maggi L
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2023;42(1):2-13
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2023;42(1):2-13
Sedimentation Assays to Assess the Impact of Posttranslational Modifications on Phase Separation of RNA-Binding Proteins In Vitro and In Cells.
Gruijs da Silva LA, Dormann D
Methods in molecular biology (Clifton, N.J.) 2023;2563:325-339
Methods in molecular biology (Clifton, N.J.) 2023;2563:325-339
Protocol for the Systematic Fixation, Circuit-Based Sampling, and Qualitative and Quantitative Neuropathological Analysis of Human Brain Tissue.
Latimer CS, Melief EJ, Ariza-Torres J, Howard K, Keen AR, Keene LM, Schantz AM, Sytsma TM, Wilson AM, Grabowski TJ, Darvas M, O'Connor KD, Nolan AL, Edlow BL, Mac Donald CL, Keene CD
Methods in molecular biology (Clifton, N.J.) 2023;2561:3-30
Methods in molecular biology (Clifton, N.J.) 2023;2561:3-30
Neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis.
Pisciottani A, Croci L, Lauria F, Marullo C, Savino E, Ambrosi A, Podini P, Marchioretto M, Casoni F, Cremona O, Taverna S, Quattrini A, Cioni JM, Viero G, Codazzi F, Consalez GG
Frontiers in cellular neuroscience 2023;17:1253543
Frontiers in cellular neuroscience 2023;17:1253543
Loss of TDP-43 function contributes to genomic instability in amyotrophic lateral sclerosis.
Fang M, Deibler SK, Nana AL, Vatsavayai SC, Banday S, Zhou Y, Almeida S, Weiss A, Brown RH, Seeley WW, Gao FB, Green MR
Frontiers in neuroscience 2023;17:1251228
Frontiers in neuroscience 2023;17:1251228
TTBK1 and CK1 inhibitors restore TDP-43 pathology and avoid disease propagation in lymphoblast from Alzheimer's disease patients.
Martinez-Gonzalez L, Cuevas EP, Tosat-Bitrián C, Nozal V, Gil C, Palomo V, Martín-Requero Á, Martinez A
Frontiers in molecular neuroscience 2023;16:1243277
Frontiers in molecular neuroscience 2023;16:1243277
Codon-optimized TDP-43 mediates neurodegeneration in a Drosophila model of ALS/FTLD.
Yusuff T, Chang YC, Sang TK, Jackson GR, Chatterjee S
Frontiers in genetics 2023;14:881638
Frontiers in genetics 2023;14:881638
Differential effects on TDP-43, piezo-2, tight-junction proteins in various brain regions following repetitive low-intensity blast overpressure.
Heyburn L, Dahal S, Abutarboush R, Reed E, Urioste R, Batuure A, Wilder D, Ahlers ST, Long JB, Sajja VS
Frontiers in neurology 2023;14:1237647
Frontiers in neurology 2023;14:1237647
The identification of high-performing antibodies for TDP-43 for use in Western Blot, immunoprecipitation and immunofluorescence.
Worrall D, Ayoubi R, Fotouhi M, Southern K, McPherson PS, Laflamme C, NeuroSGC/YCharOS/EDDU collaborative group, ABIF Consortium
F1000Research 2023;12:277
F1000Research 2023;12:277
Increased oligomeric TDP-43 in the plasma of Korean frontotemporal dementia patients with semantic dementia.
Jamerlan AM, Shim KH, Youn YC, Teunissen C, An SSA, Scheltens P, Kim S
Alzheimer's & dementia : the journal of the Alzheimer's Association 2023 Sep;19(9):4020-4027
Alzheimer's & dementia : the journal of the Alzheimer's Association 2023 Sep;19(9):4020-4027
A panel of TDP-43-regulated splicing events verifies loss of TDP-43 function in amyotrophic lateral sclerosis brain tissue.
Cao MC, Ryan B, Wu J, Curtis MA, Faull RLM, Dragunow M, Scotter EL
Neurobiology of disease 2023 Sep;185:106245
Neurobiology of disease 2023 Sep;185:106245
Loss of TDP-43 oligomerization or RNA binding elicits distinct aggregation patterns.
Pérez-Berlanga M, Wiersma VI, Zbinden A, De Vos L, Wagner U, Foglieni C, Mallona I, Betz KM, Cléry A, Weber J, Guo Z, Rigort R, de Rossi P, Manglunia R, Tantardini E, Sahadevan S, Stach O, Hruska-Plochan M, Allain FH, Paganetti P, Polymenidou M
The EMBO journal 2023 Sep 4;42(17):e111719
The EMBO journal 2023 Sep 4;42(17):e111719
Su(var)3-9 mediates age-dependent increase in H3K9 methylation on TDP-43 promoter triggering neurodegeneration.
Marzullo M, Romano G, Pellacani C, Riccardi F, Ciapponi L, Feiguin F
Cell death discovery 2023 Sep 27;9(1):357
Cell death discovery 2023 Sep 27;9(1):357
iPSC motor neurons, but not other derived cell types, capture gene expression changes in postmortem sporadic ALS motor neurons.
Held A, Adler M, Marques C, Reyes CJ, Kavuturu AS, Quadros ARAA, Ndayambaje IS, Lara E, Ward M, Lagier-Tourenne C, Wainger BJ
Cell reports 2023 Sep 26;42(9):113046
Cell reports 2023 Sep 26;42(9):113046
Causal ALS genes impact the MHC class II antigen presentation pathway.
Chi B, Öztürk MM, Paraggio CL, Leonard CE, Sanita ME, Dastpak M, O'Connell JD, Coady JA, Zhang J, Gygi SP, Lopez-Gonzalez R, Yin S, Reed R
Proceedings of the National Academy of Sciences of the United States of America 2023 Sep 26;120(39):e2305756120
Proceedings of the National Academy of Sciences of the United States of America 2023 Sep 26;120(39):e2305756120
Cytotoxic immune cells do not affect TDP-43 and p62 sarcoplasmic aggregation but influence TDP-43 localisation.
McCord B, Day RM
Scientific reports 2023 Sep 23;13(1):15935
Scientific reports 2023 Sep 23;13(1):15935
TDP43 pathology in chronic traumatic encephalopathy retinas.
Phansalkar R, Goodwill VS, Nirschl JJ, De Lillo C, Choi J, Spurlock E, Coughlin DG, Pizzo D, Sigurdson CJ, Hiniker A, Alvarez VE, Mckee AC, Lin JH
Acta neuropathologica communications 2023 Sep 22;11(1):152
Acta neuropathologica communications 2023 Sep 22;11(1):152
Tuning Hsp104 specificity to selectively detoxify α-synuclein.
Mack KL, Kim H, Barbieri EM, Lin J, Braganza S, Jackrel ME, DeNizio JE, Yan X, Chuang E, Tariq A, Cupo RR, Castellano LM, Caldwell KA, Caldwell GA, Shorter J
Molecular cell 2023 Sep 21;83(18):3314-3332.e9
Molecular cell 2023 Sep 21;83(18):3314-3332.e9
Selective Noradrenaline Depletion in the Neocortex and Hippocampus Induces Working Memory Deficits and Regional Occurrence of Pathological Proteins.
Prinzi C, Kostenko A, de Leo G, Gulino R, Leanza G, Caccamo A
Biology 2023 Sep 21;12(9)
Biology 2023 Sep 21;12(9)
Rare exonic variant affects GRN splicing and contributes to frontotemporal lobar degeneration.
Wauters E, Gossye H, Frydas A, Sieben A, Van Broeckhoven C
Neurobiology of aging 2023 Oct;130:61-69
Neurobiology of aging 2023 Oct;130:61-69
Human herpesvirus 8 ORF57 protein is able to reduce TDP-43 pathology: network analysis identifies interacting pathways.
Webber CJ, Murphy CN, Rondón-Ortiz AN, van der Spek SJF, Kelly EX, Lampl NM, Chiesa G, Khalil AS, Emili A, Wolozin B
Human molecular genetics 2023 Oct 4;32(20):2966-2980
Human molecular genetics 2023 Oct 4;32(20):2966-2980
circPSD3 is a promising inhibitor of uPA system to inhibit vascular invasion and metastasis in hepatocellular carcinoma.
Xu L, Wang P, Li L, Li L, Huang Y, Zhang Y, Zheng X, Yi P, Zhang M, Xu M
Molecular cancer 2023 Oct 26;22(1):174
Molecular cancer 2023 Oct 26;22(1):174
Amyloidogenic propensity of self-assembling peptides and their adjuvant potential for use as DNA vaccines.
Shrimali PC, Chen S, Das A, Dreher R, Howard MK, Ryan JJ, Buck J, Kim D, Sprunger ML, Rudra JS, Jackrel ME
Acta biomaterialia 2023 Oct 1;169:464-476
Acta biomaterialia 2023 Oct 1;169:464-476
A review on neurodegeneration in the fast-ageing killifish, the first animal model to study the natural occurrence of neuronal cell loss.
Bergmans S, Raes L, Moons L, De Groef L
Ageing research reviews 2023 Nov;91:102065
Ageing research reviews 2023 Nov;91:102065
m(1)A in CAG repeat RNA binds to TDP-43 and induces neurodegeneration.
Sun Y, Dai H, Dai X, Yin J, Cui Y, Liu X, Gonzalez G, Yuan J, Tang F, Wang N, Perlegos AE, Bonini NM, Yang XW, Gu W, Wang Y
Nature 2023 Nov;623(7987):580-587
Nature 2023 Nov;623(7987):580-587
Different Chronic Stress Paradigms Converge on Endogenous TDP43 Cleavage and Aggregation.
Candelise N, Caissutti D, Zenuni H, Nesci V, Scaricamazza S, Salvatori I, Spinello Z, Mattei V, Garofalo T, Ferri A, Valle C, Misasi R
Molecular neurobiology 2023 Nov;60(11):6346-6361
Molecular neurobiology 2023 Nov;60(11):6346-6361
Dysregulation of the progranulin-driven autophagy-lysosomal pathway mediates secretion of the nuclear protein TDP-43.
Tanaka Y, Ito SI, Honma Y, Hasegawa M, Kametani F, Suzuki G, Kozuma L, Takeya K, Eto M
The Journal of biological chemistry 2023 Nov;299(11):105272
The Journal of biological chemistry 2023 Nov;299(11):105272
Chronic exposure to l-BMAA cyanotoxin induces cytoplasmic TDP-43 accumulation and glial activation, reproducing an amyotrophic lateral sclerosis-like phenotype in mice.
Anzilotti S, Valente V, Brancaccio P, Franco C, Casamassa A, Lombardi G, Palazzi A, Conte A, Paladino S, Canzoniero LMT, Annunziato L, Pierantoni GM, Pignataro G
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie 2023 Nov;167:115503
Biomedicine & pharmacotherapy = Biomedecine & pharmacotherapie 2023 Nov;167:115503
G(2)C(4) targeting antisense oligonucleotides potently mitigate TDP-43 dysfunction in human C9orf72 ALS/FTD induced pluripotent stem cell derived neurons.
Rothstein JD, Baskerville V, Rapuri S, Mehlhop E, Jafar-Nejad P, Rigo F, Bennett F, Mizielinska S, Isaacs A, Coyne AN
Acta neuropathologica 2023 Nov 29;147(1):1
Acta neuropathologica 2023 Nov 29;147(1):1
Time-resolved proteomic profiling reveals compositional and functional transitions across the stress granule life cycle.
Hu S, Zhang Y, Yi Q, Yang C, Liu Y, Bai Y
Nature communications 2023 Nov 27;14(1):7782
Nature communications 2023 Nov 27;14(1):7782
Targeted perturbation of signaling-driven condensates.
Gui T, Fleming C, Manzato C, Bourgeois B, Sirati N, Heuer J, Papadionysiou I, Montfort DIV, Gijzen MV, Smits LMM, Burgering BMT, Madl T, Schuijers J
Molecular cell 2023 Nov 16;83(22):4141-4157.e11
Molecular cell 2023 Nov 16;83(22):4141-4157.e11
Creation of de novo cryptic splicing for ALS/FTD precision medicine.
Wilkins OG, Chien MZYJ, Wlaschin JJ, Pisliakova M, Thompson D, Digby H, Simkin RL, Diaz JA, Mehta PR, Keuss MJ, Zanovello M, Brown AL, Harley P, Darbey A, Karda R, Fisher EMC, Cunningham TJ, Le Pichon CE, Ule J, Fratta P
bioRxiv : the preprint server for biology 2023 Nov 15;
bioRxiv : the preprint server for biology 2023 Nov 15;
TDP-43 knockdown in mouse model of ALS leads to dsRNA deposition, gliosis, and neurodegeneration in the spinal cord.
Milstead RA, Link CD, Xu Z, Hoeffer CA
Cerebral cortex (New York, N.Y. : 1991) 2023 May 9;33(10):5808-5816
Cerebral cortex (New York, N.Y. : 1991) 2023 May 9;33(10):5808-5816
Preclinical investigation in FAAH inhibition as a neuroprotective therapy for frontotemporal dementia using TDP-43 transgenic male mice.
Santos-García I, Rodríguez-Cueto C, Villegas P, Piscitelli F, Lauritano A, Shen CJ, Di Marzo V, Fernández-Ruiz J, de Lago E
Journal of neuroinflammation 2023 May 6;20(1):108
Journal of neuroinflammation 2023 May 6;20(1):108
A New Mouse Model of Giant Axonal Neuropathy with Overt Phenotypes and Neurodegeneration Driven by Neurofilament Disorganization.
Nath B, Julien JP
The Journal of neuroscience : the official journal of the Society for Neuroscience 2023 May 31;43(22):4174-4189
The Journal of neuroscience : the official journal of the Society for Neuroscience 2023 May 31;43(22):4174-4189
Looking for answers far away from the soma-the (un)known axonal functions of TDP-43, and their contribution to early NMJ disruption in ALS.
Ionescu A, Altman T, Perlson E
Molecular neurodegeneration 2023 May 31;18(1):35
Molecular neurodegeneration 2023 May 31;18(1):35
Tandem detergent-extraction and immunoprecipitation of proteinopathy: Scalable enrichment of ALS-associated TDP-43 aggregates.
Evangelista BA, Cahalan SR, Ragusa JV, Mordant A, Necarsulmer JC, Perna RJ, Ajit T, White K, Barker NK, Tian X, Cohen S, Meeker R, Herring LE, Cohen TJ
iScience 2023 May 19;26(5):106645
iScience 2023 May 19;26(5):106645
Elevated nuclear TDP-43 induces constitutive exon skipping.
Carmen-Orozco RP, Tsao W, Ye Y, Sinha IR, Chang K, Trinh V, Chung W, Bowden K, Troncoso JC, Blackshaw S, Hayes LR, Sun S, Wong PC, Ling JP
bioRxiv : the preprint server for biology 2023 May 12;
bioRxiv : the preprint server for biology 2023 May 12;
SULT1A1-dependent sulfonation of alkylators is a lineage-dependent vulnerability of liver cancers.
Shi L, Shen W, Davis MI, Kong K, Vu P, Saha SK, Adil R, Kreuzer J, Egan R, Lee TD, Greninger P, Shrimp JH, Zhao W, Wei TY, Zhou M, Eccleston J, Sussman J, Manocha U, Weerasekara V, Kondo H, Vijay V, Wu MJ, Kearney SE, Ho J, McClanaghan J, Murchie E, Crowther GS, Patnaik S, Boxer MB, Shen M, Ting DT, Kim WY, Stanger BZ, Deshpande V, Ferrone CR, Benes CH, Haas W, Hall MD, Bardeesy N
Nature cancer 2023 Mar;4(3):365-381
Nature cancer 2023 Mar;4(3):365-381
MicroRNA-183-5p regulates TAR DNA-binding protein 43 neurotoxicity via SQSTM1/p62 in amyotrophic lateral sclerosis.
Kim HC, Zhang Y, King PH, Lu L
Journal of neurochemistry 2023 Mar;164(5):643-657
Journal of neurochemistry 2023 Mar;164(5):643-657
Accumulation of TMEM106B C-terminal fragments in neurodegenerative disease and aging.
Perneel J, Neumann M, Heeman B, Cheung S, Van den Broeck M, Wynants S, Baker M, Vicente CT, Faura J, Rademakers R, Mackenzie IRA
Acta neuropathologica 2023 Mar;145(3):285-302
Acta neuropathologica 2023 Mar;145(3):285-302
Deficiency of Perry syndrome-associated p150(Glued) in midbrain dopaminergic neurons leads to progressive neurodegeneration and endoplasmic reticulum abnormalities.
Yu J, Yang X, Zheng J, Sgobio C, Sun L, Cai H
NPJ Parkinson's disease 2023 Mar 7;9(1):35
NPJ Parkinson's disease 2023 Mar 7;9(1):35
Loss of C9orf72 perturbs the Ran-GTPase gradient and nucleocytoplasmic transport, generating compositionally diverse Importin β-1 granules.
McGoldrick P, Lau A, You Z, Durcan TM, Robertson J
Cell reports 2023 Mar 28;42(3):112134
Cell reports 2023 Mar 28;42(3):112134
Development of FRET and Stress Granule Dual-Based System to Screen for Viral 3C Protease Inhibitors.
Zhang J, Jiang Y, Wu C, Zhou D, Gong J, Zhao T, Jin Z
Molecules (Basel, Switzerland) 2023 Mar 28;28(7)
Molecules (Basel, Switzerland) 2023 Mar 28;28(7)
Aggregation-prone TDP-43 sequesters and drives pathological transitions of free nuclear TDP-43.
Keating SS, Bademosi AT, San Gil R, Walker AK
Cellular and molecular life sciences : CMLS 2023 Mar 17;80(4):95
Cellular and molecular life sciences : CMLS 2023 Mar 17;80(4):95
ILF3 prion-like domain regulates gene expression and fear memory under chronic stress.
Yamashita A, Shichino Y, Fujii K, Koshidaka Y, Adachi M, Sasagawa E, Mito M, Nakagawa S, Iwasaki S, Takao K, Shiina N
iScience 2023 Mar 17;26(3):106229
iScience 2023 Mar 17;26(3):106229
Sustained therapeutic benefits by transient reduction of TDP-43 using ENA-modified antisense oligonucleotides in ALS/FTD mice.
Takeuchi T, Maeta K, Ding X, Oe Y, Takeda A, Inoue M, Nagano S, Fujihara T, Matsuda S, Ishigaki S, Sahashi K, Minakawa EN, Mochizuki H, Neya M, Sobue G, Nagai Y
Molecular therapy. Nucleic acids 2023 Mar 14;31:353-366
Molecular therapy. Nucleic acids 2023 Mar 14;31:353-366
Ballooned neurons in semi-recent severe traumatic brain injury.
Michaud J, Plu I, Parai J, Bourgault A, Tanguay C, Seilhean D, Woulfe J
Acta neuropathologica communications 2023 Mar 10;11(1):37
Acta neuropathologica communications 2023 Mar 10;11(1):37
Macroglossia in rapidly progressive inclusion body myositis.
Yamasaki Y, Mukaino A, Yamashita S, Takeuchi Y, Tawara N, Yoshida R, Honda Y, Yamashita T, Kakimoto A, Ueyama H, Ando Y
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Jun;43(3):252-256
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Jun;43(3):252-256
Viral vector gene delivery of the novel chaperone protein SRCP1 to modify insoluble protein in in vitro and in vivo models of ALS.
Luecke IW, Lin G, Santarriaga S, Scaglione KM, Ebert AD
Gene therapy 2023 Jun;30(6):528-533
Gene therapy 2023 Jun;30(6):528-533
Early activation of cellular stress and death pathways caused by cytoplasmic TDP-43 in the rNLS8 mouse model of ALS and FTD.
Luan W, Wright AL, Brown-Wright H, Le S, San Gil R, Madrid San Martin L, Ling K, Jafar-Nejad P, Rigo F, Walker AK
Molecular psychiatry 2023 Jun;28(6):2445-2461
Molecular psychiatry 2023 Jun;28(6):2445-2461
Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage.
Kumar ST, Nazarov S, Porta S, Maharjan N, Cendrowska U, Kabani M, Finamore F, Xu Y, Lee VM, Lashuel HA
Nature neuroscience 2023 Jun;26(6):983-996
Nature neuroscience 2023 Jun;26(6):983-996
CircTmeff1 Promotes Muscle Atrophy by Interacting with TDP-43 and Encoding A Novel TMEFF1-339aa Protein.
Chen R, Yang T, Jin B, Xu W, Yan Y, Wood N, Lehmann HI, Wang S, Zhu X, Yuan W, Chen H, Liu Z, Li G, Bowen TS, Li J, Xiao J
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2023 Jun;10(17):e2206732
Advanced science (Weinheim, Baden-Wurttemberg, Germany) 2023 Jun;10(17):e2206732
Suppression of TDP-43 aggregation by artificial peptide binder targeting to its low complexity domain.
Kamagata K, Kanbayashi S, Koda S, Kadotani A, Ubukata O, Tashima T
Biochemical and biophysical research communications 2023 Jun 25;662:119-125
Biochemical and biophysical research communications 2023 Jun 25;662:119-125
Molecular and Phenotypic Changes in FLExDUX4 Mice.
Murphy K, Zhang A, Bittel AJ, Chen YW
Journal of personalized medicine 2023 Jun 25;13(7)
Journal of personalized medicine 2023 Jun 25;13(7)
The influence of age and sex on the absolute cell numbers of the human brain cerebral cortex.
Castro-Fonseca E, Morais V, da Silva CG, Wollner J, Freitas J, Mello-Neto AF, Oliveira LE, de Oliveira VC, Leite REP, Alho AT, Rodriguez RD, Ferretti-Rebustini REL, Suemoto CK, Jacob-Filho W, Nitrini R, Pasqualucci CA, Grinberg LT, Tovar-Moll F, Lent R
Cerebral cortex (New York, N.Y. : 1991) 2023 Jun 20;33(13):8654-8666
Cerebral cortex (New York, N.Y. : 1991) 2023 Jun 20;33(13):8654-8666
Disruption of ER ion homeostasis maintained by an ER anion channel CLCC1 contributes to ALS-like pathologies.
Guo L, Mao Q, He J, Liu X, Piao X, Luo L, Hao X, Yu H, Song Q, Xiao B, Fan D, Gao Z, Jia Y
Cell research 2023 Jul;33(7):497-515
Cell research 2023 Jul;33(7):497-515
Nuclear Expression of TDP-43 Is Linked with Morphology and Ubiquitylation of Cytoplasmic Aggregates in Amyotrophic Lateral Sclerosis.
Yabata H, Riku Y, Miyahara H, Akagi A, Sone J, Urushitani M, Yoshida M, Iwasaki Y
International journal of molecular sciences 2023 Jul 29;24(15)
International journal of molecular sciences 2023 Jul 29;24(15)
Aberrant dynein function promotes TDP-43 aggregation and upregulation of p62 in male mice harboring transgenic human TDP-43.
Christoforidou E, Simoes FA, Gordon D, Talbot K, Hafezparast M
Amyotrophic lateral sclerosis & frontotemporal degeneration 2023 Jul 27;:1-10
Amyotrophic lateral sclerosis & frontotemporal degeneration 2023 Jul 27;:1-10
Specific vulnerability of iPSC-derived motor neurons with TDP-43 gene mutation to oxidative stress.
Onda-Ohto A, Hasegawa-Ogawa M, Matsuno H, Shiraishi T, Bono K, Hiraki H, Kanegae Y, Iguchi Y, Okano HJ
Molecular brain 2023 Jul 26;16(1):62
Molecular brain 2023 Jul 26;16(1):62
Neuroimmune characterization of optineurin insufficiency mouse model during ageing.
Mohovic N, Peradinovic J, Markovinovic A, Cimbro R, Minic Z, Dominovic M, Jakovac H, Nimac J, Rogelj B, Munitic I
Scientific reports 2023 Jul 22;13(1):11840
Scientific reports 2023 Jul 22;13(1):11840
Targeting the glycine-rich domain of TDP-43 with antibodies prevents its aggregation in vitro and reduces neurofilament levels in vivo.
Riemenschneider H, Simonetti F, Sheth U, Katona E, Roth S, Hutten S, Farny D, Michaelsen M, Nuscher B, Schmidt MK, Flatley A, Schepers A, Gruijs da Silva LA, Zhou Q, Klopstock T, Liesz A, Arzberger T, Herms J, Feederle R, Gendron TF, Dormann D, Edbauer D
Acta neuropathologica communications 2023 Jul 11;11(1):112
Acta neuropathologica communications 2023 Jul 11;11(1):112
Atypical TDP-43 protein expression in an ALS pedigree carrying a p.Y374X truncation mutation in TARDBP.
Cooper-Knock J, Julian TH, Feneberg E, Highley JR, Sidra M, Turner MR, Talbot K, Ansorge O, Allen SP, Moll T, Shelkovnikova T, Castelli L, Hautbergue GM, Hewitt C, Kirby J, Wharton SB, Mead RJ, Shaw PJ
Brain pathology (Zurich, Switzerland) 2023 Jan;33(1):e13104
Brain pathology (Zurich, Switzerland) 2023 Jan;33(1):e13104
Neuropathology of central nervous system involvement in TTR amyloidosis.
Taipa R, Sousa L, Pinto M, Reis I, Rodrigues A, Oliveira P, Melo-Pires M, Coelho T
Acta neuropathologica 2023 Jan;145(1):113-126
Acta neuropathologica 2023 Jan;145(1):113-126
Tuberous sclerosis complex is associated with a novel human tauopathy.
Hwang JL, Perloff OS, Gaus SE, Benitez C, Alquezar C, Cosme CQ, Nana AL, Vatsavayai SC, Ramos EM, Geschwind DH, Miller BL, Kao AW, Seeley WW
Acta neuropathologica 2023 Jan;145(1):1-12
Acta neuropathologica 2023 Jan;145(1):1-12
Study of Ubiquitin Pathway Genes in a French Population with Amyotrophic Lateral Sclerosis: Focus on HECW1 Encoding the E3 Ligase NEDL1.
Haouari S, Andres CR, Lanznaster D, Marouillat S, Brulard C, Dangoumau A, Ung D, Veyrat-Durebex C, Laumonnier F, Blasco H, Couratier P, Corcia P, Vourc'h P, French ALS network FILSLAN
International journal of molecular sciences 2023 Jan 9;24(2)
International journal of molecular sciences 2023 Jan 9;24(2)
Stress granule assembly in vivo is deficient in the CNS of mutant TDP-43 ALS mice.
Dubinski A, Gagné M, Peyrard S, Gordon D, Talbot K, Vande Velde C
Human molecular genetics 2023 Jan 6;32(2):319-332
Human molecular genetics 2023 Jan 6;32(2):319-332
A fluid biomarker reveals loss of TDP-43 splicing repression in pre-symptomatic ALS.
Irwin KE, Jasin P, Braunstein KE, Sinha I, Bowden KD, Moghekar A, Oh ES, Raitcheva D, Bartlett D, Berry JD, Traynor B, Ling JP, Wong PC
bioRxiv : the preprint server for biology 2023 Jan 24;
bioRxiv : the preprint server for biology 2023 Jan 24;
Rapid nuclear deadenylation of mammalian messenger RNA.
Alles J, Legnini I, Pacelli M, Rajewsky N
iScience 2023 Jan 20;26(1):105878
iScience 2023 Jan 20;26(1):105878
RNA methylation influences TDP43 binding and disease pathogenesis in models of amyotrophic lateral sclerosis and frontotemporal dementia.
McMillan M, Gomez N, Hsieh C, Bekier M, Li X, Miguez R, Tank EMH, Barmada SJ
Molecular cell 2023 Jan 19;83(2):219-236.e7
Molecular cell 2023 Jan 19;83(2):219-236.e7
Amyotrophic lateral sclerosis with TDP-43 abnormalities exhibiting globular glial tau inclusions in frontotemporal lobes and pallido-nigral system.
Yagita K, Sasagasako N, Koyama S, Noguchi H, Honda H
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Feb;43(1):117-126
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Feb;43(1):117-126
Loss of TDP-43 function underlies hippocampal and cortical synaptic deficits in TDP-43 proteinopathies.
Ni J, Ren Y, Su T, Zhou J, Fu C, Lu Y, Li D, Zhao J, Li Y, Zhang Y, Fang Y, Liu N, Geng Y, Chen Y
Molecular psychiatry 2023 Feb;28(2):931-945
Molecular psychiatry 2023 Feb;28(2):931-945
Quantitative proteomics of tau and Aβ in detergent fractions from Alzheimer's disease brains.
Mukherjee S, Dubois C, Perez K, Varghese S, Birchall IE, Leckey M, Davydova N, McLean C, Nisbet RM, Roberts BR, Li QX, Masters CL, Streltsov VA
Journal of neurochemistry 2023 Feb;164(4):529-552
Journal of neurochemistry 2023 Feb;164(4):529-552
Distinctive chaperonopathy in skeletal muscle associated with the dominant variant in DNAJB4.
Inoue M, Noguchi S, Inoue YU, Iida A, Ogawa M, Bengoechea R, Pittman SK, Hayashi S, Watanabe K, Hosoi Y, Sano T, Takao M, Oya Y, Takahashi Y, Miyajima H, Weihl CC, Inoue T, Nishino I
Acta neuropathologica 2023 Feb;145(2):235-255
Acta neuropathologica 2023 Feb;145(2):235-255
Progranulin Deficiency Induces Mitochondrial Dysfunction in Frontotemporal Lobar Degeneration with TDP-43 Inclusions.
Rodríguez-Periñán G, de la Encarnación A, Moreno F, López de Munain A, Martínez A, Martín-Requero Á, Alquézar C, Bartolomé F
Antioxidants (Basel, Switzerland) 2023 Feb 25;12(3)
Antioxidants (Basel, Switzerland) 2023 Feb 25;12(3)
Endogenous retroviruses and TDP-43 proteinopathy form a sustaining feedback driving intercellular spread of Drosophila neurodegeneration.
Chang YH, Dubnau J
Nature communications 2023 Feb 21;14(1):966
Nature communications 2023 Feb 21;14(1):966
SYF2 suppression mitigates neurodegeneration in models of diverse forms of ALS.
Linares GR, Li Y, Chang WH, Rubin-Sigler J, Mendonca S, Hong S, Eoh Y, Guo W, Huang YH, Chang J, Tu S, Dorjsuren N, Santana M, Hung ST, Yu J, Perez J, Chickering M, Cheng TY, Huang CC, Lee SJ, Deng HJ, Bach KT, Gray K, Subramanyam V, Rosenfeld J, Alworth SV, Goodarzi H, Ichida JK
Cell stem cell 2023 Feb 2;30(2):171-187.e14
Cell stem cell 2023 Feb 2;30(2):171-187.e14
Functional Characterization of a Familial ALS-Associated Missense TBK1 (p-Arg573Gly) Mutation in Patient-Derived Lymphoblasts.
Porras G, Ruiz S, Maestro I, Borrego-Hernández D, Redondo AG, Martínez A, Martín-Requero Á
International journal of molecular sciences 2023 Feb 2;24(3)
International journal of molecular sciences 2023 Feb 2;24(3)
MATR3 P154S knock-in mice do not exhibit motor, muscle or neuropathologic features of ALS.
Dominick M, Houchins N, Venugopal V, Zuberi AR, Lutz CM, Meechooveet B, Van Keuren-Jensen K, Bowser R, Medina DX
Biochemical and biophysical research communications 2023 Feb 19;645:164-172
Biochemical and biophysical research communications 2023 Feb 19;645:164-172
PIKFYVE inhibition mitigates disease in models of diverse forms of ALS.
Hung ST, Linares GR, Chang WH, Eoh Y, Krishnan G, Mendonca S, Hong S, Shi Y, Santana M, Kueth C, Macklin-Isquierdo S, Perry S, Duhaime S, Maios C, Chang J, Perez J, Couto A, Lai J, Li Y, Alworth SV, Hendricks E, Wang Y, Zlokovic BV, Dickman DK, Parker JA, Zarnescu DC, Gao FB, Ichida JK
Cell 2023 Feb 16;186(4):786-802.e28
Cell 2023 Feb 16;186(4):786-802.e28
Ataxin-2 polyglutamine expansions aberrantly sequester TDP-43, drive ribonucleoprotein condensate transport dysfunction and suppress local translation.
Wijegunawardana D, Vishal SS, Venkatesh N, Gopal PP
bioRxiv : the preprint server for biology 2023 Feb 15;
bioRxiv : the preprint server for biology 2023 Feb 15;
Exosomal TAR DNA binding protein 43 profile in canine model of amyotrophic lateral sclerosis: a preliminary study in developing blood-based biomarker for neurodegenerative diseases.
Pfeiffer P, Coates JR, Esqueda YM, Kennedy A, Getchell K, McLenon M, Kosa E, Agbas A
Annals of medicine 2023 Dec;55(1):34-41
Annals of medicine 2023 Dec;55(1):34-41
An Autopsy Series of Seven Cases of VPS13A Disease (Chorea-Acanthocytosis).
Ditzel RM Jr, Walker RH, Nirenberg MJ, Tetlow AM, Farrell K, Lind-Watson KJ, Thorn EL, Dangoor DK, Gordon R, De Sanctis C, Barton B, Karp BI, Kirby A, Lett DJ, Mente K, Simon DK, Velayos-Baeza A, Miltenberger-Miltenyi G, Humphrey J, Crary JF
Movement disorders : official journal of the Movement Disorder Society 2023 Dec;38(12):2163-2172
Movement disorders : official journal of the Movement Disorder Society 2023 Dec;38(12):2163-2172
GM1 ganglioside exerts protective effects against glutamate-excitotoxicity via its oligosaccharide in wild-type and amyotrophic lateral sclerosis motor neurons.
Lunghi G, Di Biase E, Carsana EV, Henriques A, Callizot N, Mauri L, Ciampa MG, Mari L, Loberto N, Aureli M, Sonnino S, Spedding M, Chiricozzi E, Fazzari M
FEBS open bio 2023 Dec;13(12):2324-2341
FEBS open bio 2023 Dec;13(12):2324-2341
Aberrant axon initial segment plasticity and intrinsic excitability of ALS hiPSC motor neurons.
Harley P, Kerins C, Gatt A, Neves G, Riccio F, Machado CB, Cheesbrough A, R'Bibo L, Burrone J, Lieberam I
Cell reports 2023 Dec 26;42(12):113509
Cell reports 2023 Dec 26;42(12):113509
Loss of TDP-43 splicing repression occurs early in the aging population and is associated with Alzheimer's disease neuropathologic changes and cognitive decline.
Chang K, Ling JP, Redding-Ochoa J, An Y, Li L, Dean SA, Blanchard TG, Pylyukh T, Barrett A, Irwin KE, Moghekar A, Resnick SM, Wong PC, Troncoso JC
Acta neuropathologica 2023 Dec 22;147(1):4
Acta neuropathologica 2023 Dec 22;147(1):4
Highly variable molecular signatures of TDP-43 loss of function are associated with nuclear pore complex injury in a population study of sporadic ALS patient iPSNs.
Rothstein JD, Warlick C, Coyne AN
bioRxiv : the preprint server for biology 2023 Dec 13;
bioRxiv : the preprint server for biology 2023 Dec 13;
Repeated mild traumatic brain injury triggers pathology in asymptomatic C9ORF72 transgenic mice.
Kahriman A, Bouley J, Tuncali I, Dogan EO, Pereira M, Luu T, Bosco DA, Jaber S, Peters OM, Brown RH Jr, Henninger N
Brain : a journal of neurology 2023 Dec 1;146(12):5139-5152
Brain : a journal of neurology 2023 Dec 1;146(12):5139-5152
TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP.
Arseni D, Chen R, Murzin AG, Peak-Chew SY, Garringer HJ, Newell KL, Kametani F, Robinson AC, Vidal R, Ghetti B, Hasegawa M, Ryskeldi-Falcon B
Nature 2023 Aug;620(7975):898-903
Nature 2023 Aug;620(7975):898-903
Neuromuscular junction denervation and terminal Schwann cell loss in the hTDP-43 overexpression mouse model of amyotrophic lateral sclerosis.
Alhindi A, Shand M, Smith HL, Leite AS, Huang YT, van der Hoorn D, Ridgway Z, Faller KME, Jones RA, Gillingwater TH, Chaytow H
Neuropathology and applied neurobiology 2023 Aug;49(4):e12925
Neuropathology and applied neurobiology 2023 Aug;49(4):e12925
Psychotic symptoms in frontotemporal dementia with TDP-43 tend to be associated with type B pathology.
Hirsch-Reinshagen V, Hercher C, Vila-Rodriguez F, Neumann M, Rademakers R, Honer WG, Hsiung GR, Mackenzie IR
Neuropathology and applied neurobiology 2023 Aug;49(4):e12921
Neuropathology and applied neurobiology 2023 Aug;49(4):e12921
Comparison of TRIBE and STAMP for identifying targets of RNA binding proteins in human and Drosophila cells.
Abruzzi KC, Ratner C, Rosbash M
RNA (New York, N.Y.) 2023 Aug;29(8):1230-1242
RNA (New York, N.Y.) 2023 Aug;29(8):1230-1242
N protein of PEDV plays chess game with host proteins by selective autophagy.
Zhai X, Kong N, Zhang Y, Song Y, Qin W, Yang X, Ye C, Ye M, Tong W, Liu C, Zheng H, Yu H, Zhang W, Yang X, Zhang G, Tong G, Shan T
Autophagy 2023 Aug;19(8):2338-2352
Autophagy 2023 Aug;19(8):2338-2352
Chaperone-mediated autophagy in neuronal dendrites utilizes activity-dependent lysosomal exocytosis for protein disposal.
Grochowska KM, Sperveslage M, Raman R, Failla AV, Głów D, Schulze C, Laprell L, Fehse B, Kreutz MR
Cell reports 2023 Aug 29;42(8):112998
Cell reports 2023 Aug 29;42(8):112998
Astrocytic response mediated by the CLU risk allele inhibits OPC proliferation and myelination in a human iPSC model.
Liu Z, Chao J, Wang C, Sun G, Roeth D, Liu W, Chen X, Li L, Tian E, Feng L, Davtyan H, Blurton-Jones M, Kalkum M, Shi Y
Cell reports 2023 Aug 29;42(8):112841
Cell reports 2023 Aug 29;42(8):112841
Traumatic brain injury induces TDP-43 mislocalization and neurodegenerative effects in tissue distal to the primary injury site in a non-transgenic mouse.
Bjorklund GR, Wong J, Brafman D, Bowser R, Stabenfeldt SE
Acta neuropathologica communications 2023 Aug 22;11(1):137
Acta neuropathologica communications 2023 Aug 22;11(1):137
Reduction of nemo-like kinase increases lysosome biogenesis and ameliorates TDP-43-related neurodegeneration.
Tejwani L, Jung Y, Kokubu H, Sowmithra S, Ni L, Lee C, Sanders B, Lee PJ, Xiang Y, Luttik K, Soriano A, Yoon J, Park J, Ro HH, Ju H, Liao C, Tieze SM, Rigo F, Jafar-Nejad P, Lim J
The Journal of clinical investigation 2023 Aug 15;133(16)
The Journal of clinical investigation 2023 Aug 15;133(16)
Hyperphosphorylated Tau in Mesial Temporal Lobe Epilepsy: a Neuropathological and Cognitive Study.
Toscano ECB, Vieira ÉLM, Grinberg LT, Rocha NP, Brant JAS, Paradela RS, Giannetti AV, Suemoto CK, Leite REP, Nitrini R, Rachid MA, Teixeira AL
Molecular neurobiology 2023 Apr;60(4):2174-2185
Molecular neurobiology 2023 Apr;60(4):2174-2185
TDP-43 pathology and functional deficits in wild-type and ALS/FTD mutant cyclin F mouse models.
van Hummel A, Sabale M, Przybyla M, van der Hoven J, Chan G, Feiten AF, Chung RS, Ittner LM, Ke YD
Neuropathology and applied neurobiology 2023 Apr;49(2):e12902
Neuropathology and applied neurobiology 2023 Apr;49(2):e12902
Severe cerebrovascular pathology of the first supercentenarian to be autopsied in the world.
Iwase T, Yoshida M, Hashizume Y, Inagaki T, Iwasaki Y
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Apr;43(2):181-189
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Apr;43(2):181-189
Autophagic vacuolar myopathy involving the phenotype of spinocerebellar ataxia type 3.
Li J, Peng Y, Tang J, Li M, Zhu M, Zhou M, Fang P, Hong D
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Apr;43(2):135-142
Neuropathology : official journal of the Japanese Society of Neuropathology 2023 Apr;43(2):135-142
Astrocytic TDP-43 dysregulation impairs memory by modulating antiviral pathways and interferon-inducible chemokines.
Licht-Murava A, Meadows SM, Palaguachi F, Song SC, Jackvony S, Bram Y, Zhou C, Schwartz RE, Froemke RC, Orr AL, Orr AG
Science advances 2023 Apr 21;9(16):eade1282
Science advances 2023 Apr 21;9(16):eade1282
Modulation of tau tubulin kinases (TTBK1 and TTBK2) impacts ciliogenesis.
Bashore FM, Marquez AB, Chaikuad A, Howell S, Dunn AS, Beltran AA, Smith JL, Drewry DH, Beltran AS, Axtman AD
Scientific reports 2023 Apr 14;13(1):6118
Scientific reports 2023 Apr 14;13(1):6118
Neuronal activity regulates Matrin 3 abundance and function in a calcium-dependent manner through calpain-mediated cleavage and calmodulin binding.
Malik AM, Wu JJ, Gillies CA, Doctrove QA, Li X, Huang H, Tank EHM, Shakkottai VG, Barmada S
Proceedings of the National Academy of Sciences of the United States of America 2023 Apr 11;120(15):e2206217120
Proceedings of the National Academy of Sciences of the United States of America 2023 Apr 11;120(15):e2206217120
Argyrophilic grain disease is common in older adults and may be a risk factor for suicide: a study of Japanese forensic autopsy cases.
Yoshida K, Hata Y, Ichimata S, Okada K, Nishida N
Translational neurodegeneration 2023 Apr 1;12(1):16
Translational neurodegeneration 2023 Apr 1;12(1):16
Entorhinal Subfield Vulnerability to Neurofibrillary Tangles in Aging and the Preclinical Stage of Alzheimer's Disease.
Llamas-Rodríguez J, Oltmer J, Greve DN, Williams E, Slepneva N, Wang R, Champion S, Lang-Orsini M, Fischl B, Frosch MP, van der Kouwe AJW, Augustinack JC
Journal of Alzheimer's disease : JAD 2022;87(3):1379-1399
Journal of Alzheimer's disease : JAD 2022;87(3):1379-1399
Allele-specific silencing as therapy for familial amyotrophic lateral sclerosis caused by the p.G376D TARDBP mutation.
Romano R, De Luca M, Del Fiore VS, Pecoraro M, Lattante S, Sabatelli M, La Bella V, Bucci C
Brain communications 2022;4(6):fcac315
Brain communications 2022;4(6):fcac315
TDP-43 Oligomerization and Phase Separation Properties Are Necessary for Autoregulation.
Koehler LC, Grese ZR, Bastos ACS, Mamede LD, Heyduk T, Ayala YM
Frontiers in neuroscience 2022;16:818655
Frontiers in neuroscience 2022;16:818655
DNA damage stress-induced translocation of mutant FUS proteins into cytosolic granules and screening for translocation inhibitors.
Nogami M, Sano O, Adachi-Tominari K, Hayakawa-Yano Y, Furukawa T, Iwata H, Ogi K, Okano H, Yano M
Frontiers in molecular neuroscience 2022;15:953365
Frontiers in molecular neuroscience 2022;15:953365
ATP-binding cassette transporter expression is widely dysregulated in frontotemporal dementia with TDP-43 inclusions.
Katzeff JS, Lok HC, Bhatia S, Fu Y, Halliday GM, Kim WS
Frontiers in molecular neuroscience 2022;15:1043127
Frontiers in molecular neuroscience 2022;15:1043127
C9orf72 regulates the unfolded protein response and stress granule formation by interacting with eIF2α.
Zheng W, Wang K, Wu Y, Yan G, Zhang C, Li Z, Wang L, Chen S
Theranostics 2022;12(17):7289-7306
Theranostics 2022;12(17):7289-7306
TDP-43 Pathology Exacerbates Cognitive Decline in Primary Age-Related Tauopathy.
Smirnov DS, Salmon DP, Galasko D, Edland SD, Pizzo DP, Goodwill V, Hiniker A
Annals of neurology 2022 Sep;92(3):425-438
Annals of neurology 2022 Sep;92(3):425-438
Hyperphosphorylation-Mimetic TDP-43 Drives Amyloid Formation and Possesses Neuronal Toxicity at the Oligomeric Stage.
Chiang WC, Fang YS, Lye YS, Weng TY, Ganesan K, Huang SH, Chang LY, Chou SC, Chen YR
ACS chemical neuroscience 2022 Sep 7;13(17):2599-2612
ACS chemical neuroscience 2022 Sep 7;13(17):2599-2612
Intracellular energy controls dynamics of stress-induced ribonucleoprotein granules.
Wang T, Tian X, Kim HB, Jang Y, Huang Z, Na CH, Wang J
Nature communications 2022 Sep 23;13(1):5584
Nature communications 2022 Sep 23;13(1):5584
Importance of the Q/N-rich segment for protein stability of endogenous mouse TDP-43.
Sato T, Oda K, Sakai S, Kato R, Yamamori S, Itakura M, Kodera Y, Nishizawa M, Sasaoka T, Onodera O, Yokoyama M
Scientific reports 2022 Sep 2;12(1):14923
Scientific reports 2022 Sep 2;12(1):14923
Characterizing Lewy Pathology in 231 Essential Tremor Brains From the Essential Tremor Centralized Brain Repository.
Louis ED, Iglesias-Hernandez D, Hernandez NC, Flowers X, Kuo SH, Vonsattel JPG, Faust PL
Journal of neuropathology and experimental neurology 2022 Sep 19;81(10):796-806
Journal of neuropathology and experimental neurology 2022 Sep 19;81(10):796-806
Stress induced TDP-43 mobility loss independent of stress granules.
Streit L, Kuhn T, Vomhof T, Bopp V, Ludolph AC, Weishaupt JH, Gebhardt JCM, Michaelis J, Danzer KM
Nature communications 2022 Sep 19;13(1):5480
Nature communications 2022 Sep 19;13(1):5480
Poly(ADP-ribose) promotes toxicity of C9ORF72 arginine-rich dipeptide repeat proteins.
Gao J, Mewborne QT, Girdhar A, Sheth U, Coyne AN, Punathil R, Kang BG, Dasovich M, Veire A, DeJesus Hernandez M, Liu S, Shi Z, Dafinca R, Fouquerel E, Talbot K, Kam TI, Zhang YJ, Dickson D, Petrucelli L, van Blitterswijk M, Guo L, Dawson TM, Dawson VL, Leung AKL, Lloyd TE, Gendron TF, Rothstein JD, Zhang K
Science translational medicine 2022 Sep 14;14(662):eabq3215
Science translational medicine 2022 Sep 14;14(662):eabq3215
Integrated stress response is involved in the 24(S)-hydroxycholesterol-induced unconventional cell death mechanism.
Urano Y, Osaki S, Chiba R, Noguchi N
Cell death discovery 2022 Oct 4;8(1):406
Cell death discovery 2022 Oct 4;8(1):406
Genome-wide CRISPR screen reveals v-ATPase as a drug target to lower levels of ALS protein ataxin-2.
Kim G, Nakayama L, Blum JA, Akiyama T, Boeynaems S, Chakraborty M, Couthouis J, Tassoni-Tsuchida E, Rodriguez CM, Bassik MC, Gitler AD
Cell reports 2022 Oct 25;41(4):111508
Cell reports 2022 Oct 25;41(4):111508
Impaired disassembly of the axon initial segment restricts mitochondrial entry into damaged axons.
Kiryu-Seo S, Matsushita R, Tashiro Y, Yoshimura T, Iguchi Y, Katsuno M, Takahashi R, Kiyama H
The EMBO journal 2022 Oct 17;41(20):e110486
The EMBO journal 2022 Oct 17;41(20):e110486
Pathological 25 kDa C-Terminal Fragments of TDP-43 Are Present in Lymphoblastoid Cell Lines and Extracellular Vesicles from Patients Affected by Frontotemporal Lobar Degeneration and Neuronal Ceroidolipofuscinosis Carrying a GRN Mutation.
Cimini S, Bellini S, Saraceno C, Benussi L, Ghidoni R, Giliani SC, Puoti G, Canafoglia L, Giaccone G, Rossi G
International journal of molecular sciences 2022 Nov 9;23(22)
International journal of molecular sciences 2022 Nov 9;23(22)
Robust Generation of Ready-to-Use Cryopreserved Motor Neurons from Human Pluripotent Stem Cells for Disease Modeling.
Ting HC, Su HL, Chen MF, Harn HJ, Lin SZ, Chiou TW, Chang CY
International journal of molecular sciences 2022 Nov 3;23(21)
International journal of molecular sciences 2022 Nov 3;23(21)
Understanding In Vitro Pathways to Drug Discovery for TDP-43 Proteinopathies.
Cheng HWA, Callis TB, Montgomery AP, Danon JJ, Jorgensen WT, Ke YD, Ittner LM, Werry EL, Kassiou M
International journal of molecular sciences 2022 Nov 25;23(23)
International journal of molecular sciences 2022 Nov 25;23(23)
Ubiquitin-positive astrogliopathy clinically mimicking Parkinson's disease.
Morris M, Moghekar A, Guo H, Pletnikova O, Redding-Ochoa J, Albert M, Resnick SM, Chen L
Acta neuropathologica communications 2022 Nov 14;10(1):164
Acta neuropathologica communications 2022 Nov 14;10(1):164
Perception of Fragmented Letters by Patients With Pathologically Confirmed Dementia With Lewy Bodies or Alzheimer Disease.
Salmon DP, Smirnov DS, Coughlin DG, Hamilton JM, Landy KM, Filoteo JV, Hiniker A, Hansen LA, Galasko D
Neurology 2022 Nov 1;99(18):e2034-e2043
Neurology 2022 Nov 1;99(18):e2034-e2043
Pathogenic Mutation of TDP-43 Impairs RNA Processing in a Cell Type-Specific Manner: Implications for the Pathogenesis of ALS/FTLD.
Imaizumi K, Ideno H, Sato T, Morimoto S, Okano H
eNeuro 2022 May-Jun;9(3)
eNeuro 2022 May-Jun;9(3)
Insulin-like growth factor 5 associates with human Aß plaques and promotes cognitive impairment.
Rauskolb S, Andreska T, Fries S, von Collenberg CR, Blum R, Monoranu CM, Villmann C, Sendtner M
Acta neuropathologica communications 2022 May 5;10(1):68
Acta neuropathologica communications 2022 May 5;10(1):68
TDP43 promotes stemness of breast cancer stem cells through CD44 variant splicing isoforms.
Guo L, Ke H, Zhang H, Zou L, Yang Q, Lu X, Zhao L, Jiao B
Cell death & disease 2022 May 3;13(5):428
Cell death & disease 2022 May 3;13(5):428
TARDBP Inhibits Porcine Epidemic Diarrhea Virus Replication through Degrading Viral Nucleocapsid Protein and Activating Type I Interferon Signaling.
Dong S, Kong N, Zhang Y, Li Y, Sun D, Qin W, Zhai H, Zhai X, Yang X, Ye C, Ye M, Liu C, Yu L, Zheng H, Tong W, Yu H, Zhang W, Tong G, Shan T
Journal of virology 2022 May 25;96(10):e0007022
Journal of virology 2022 May 25;96(10):e0007022
Nuclear RNA transcript levels modulate nucleocytoplasmic distribution of ALS/FTD-associated protein FUS.
Tsai YL, Mu YC, Manley JL
Scientific reports 2022 May 17;12(1):8180
Scientific reports 2022 May 17;12(1):8180
Heat shock protein Grp78/BiP/HspA5 binds directly to TDP-43 and mitigates toxicity associated with disease pathology.
François-Moutal L, Scott DD, Ambrose AJ, Zerio CJ, Rodriguez-Sanchez M, Dissanayake K, May DG, Carlson JM, Barbieri E, Moutal A, Roux KJ, Shorter J, Khanna R, Barmada SJ, McGurk L, Khanna M
Scientific reports 2022 May 17;12(1):8140
Scientific reports 2022 May 17;12(1):8140
Nuclear pore destruction and loss of nuclear TDP-43 in FUS mutation-related amyotrophic lateral sclerosis motor neurons.
Aizawa H, Teramoto S, Hideyama T, Kato H, Terashi H, Suzuki Y, Kimura T, Kwak S
Journal of the neurological sciences 2022 May 15;436:120187
Journal of the neurological sciences 2022 May 15;436:120187
TDP-43 regulates cholesterol biosynthesis by inhibiting sterol regulatory element-binding protein 2.
Egawa N, Izumi Y, Suzuki H, Tsuge I, Fujita K, Shimano H, Izumikawa K, Takahashi N, Tsukita K, Enami T, Nakamura M, Watanabe A, Naitoh M, Suzuki S, Seki T, Kobayashi K, Toda T, Kaji R, Takahashi R, Inoue H
Scientific reports 2022 May 14;12(1):7988
Scientific reports 2022 May 14;12(1):7988
TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A.
Brown AL, Wilkins OG, Keuss MJ, Hill SE, Zanovello M, Lee WC, Bampton A, Lee FCY, Masino L, Qi YA, Bryce-Smith S, Gatt A, Hallegger M, Fagegaltier D, Phatnani H, NYGC ALS Consortium, Newcombe J, Gustavsson EK, Seddighi S, Reyes JF, Coon SL, Ramos D, Schiavo G, Fisher EMC, Raj T, Secrier M, Lashley T, Ule J, Buratti E, Humphrey J, Ward ME, Fratta P
Nature 2022 Mar;603(7899):131-137
Nature 2022 Mar;603(7899):131-137
TDP-43 represses cryptic exon inclusion in the FTD-ALS gene UNC13A.
Ma XR, Prudencio M, Koike Y, Vatsavayai SC, Kim G, Harbinski F, Briner A, Rodriguez CM, Guo C, Akiyama T, Schmidt HB, Cummings BB, Wyatt DW, Kurylo K, Miller G, Mekhoubad S, Sallee N, Mekonnen G, Ganser L, Rubien JD, Jansen-West K, Cook CN, Pickles S, Oskarsson B, Graff-Radford NR, Boeve BF, Knopman DS, Petersen RC, Dickson DW, Shorter J, Myong S, Green EM, Seeley WW, Petrucelli L, Gitler AD
Nature 2022 Mar;603(7899):124-130
Nature 2022 Mar;603(7899):124-130
Sirtuin-1 sensitive lysine-136 acetylation drives phase separation and pathological aggregation of TDP-43.
Garcia Morato J, Hans F, von Zweydorf F, Feederle R, Elsässer SJ, Skodras AA, Gloeckner CJ, Buratti E, Neumann M, Kahle PJ
Nature communications 2022 Mar 9;13(1):1223
Nature communications 2022 Mar 9;13(1):1223
RTL8 promotes nuclear localization of UBQLN2 to subnuclear compartments associated with protein quality control.
Mohan HM, Trzeciakiewicz H, Pithadia A, Crowley EV, Pacitto R, Safren N, Trotter B, Zhang C, Zhou X, Zhang Y, Basrur V, Paulson HL, Sharkey LM
Cellular and molecular life sciences : CMLS 2022 Mar 5;79(3):176
Cellular and molecular life sciences : CMLS 2022 Mar 5;79(3):176
Accumulation of Nonfibrillar TDP-43 in the Rough Endoplasmic Reticulum Is the Early-Stage Pathology in Amyotrophic Lateral Sclerosis.
Kon T, Mori F, Tanji K, Miki Y, Nishijima H, Nakamura T, Kinoshita I, Suzuki C, Kurotaki H, Tomiyama M, Wakabayashi K
Journal of neuropathology and experimental neurology 2022 Mar 29;81(4):271-281
Journal of neuropathology and experimental neurology 2022 Mar 29;81(4):271-281
Therapeutic modulation of GSTO activity rescues FUS-associated neurotoxicity via deglutathionylation in ALS disease models.
Cha SJ, Lee S, Choi HJ, Han YJ, Jeon YM, Jo M, Lee S, Nahm M, Lim SM, Kim SH, Kim HJ, Kim K
Developmental cell 2022 Mar 28;57(6):783-798.e8
Developmental cell 2022 Mar 28;57(6):783-798.e8
An open source toolkit for repurposing Illumina sequencing systems as versatile fluidics and imaging platforms.
Pandit K, Petrescu J, Cuevas M, Stephenson W, Smibert P, Phatnani H, Maniatis S
Scientific reports 2022 Mar 24;12(1):5081
Scientific reports 2022 Mar 24;12(1):5081
ADARs act as potent regulators of circular transcriptome in cancer.
Shen H, An O, Ren X, Song Y, Tang SJ, Ke XY, Han J, Tay DJT, Ng VHE, Molias FB, Pitcheshwar P, Leong KW, Tan KK, Yang H, Chen L
Nature communications 2022 Mar 21;13(1):1508
Nature communications 2022 Mar 21;13(1):1508
Parallel Appearance of Polyglutamine and Transactivation-Responsive DNA-Binding Protein 43 and Their Complementary Subcellular Localization in Brains of Patients With Spinocerebellar Ataxia Type 2.
Koyano S, Yagishita S, Tada M, Doi H, Uchihara T, Tanaka F
Journal of neuropathology and experimental neurology 2022 Jun 20;81(7):535-544
Journal of neuropathology and experimental neurology 2022 Jun 20;81(7):535-544
Optineurin Deficiency and Insufficiency Lead to Higher Microglial TDP-43 Protein Levels.
Prtenjaca N, Rob M, Alam MS, Markovinovic A, Stuani C, Buratti E, Munitic I
International journal of molecular sciences 2022 Jun 19;23(12)
International journal of molecular sciences 2022 Jun 19;23(12)
NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility.
Gleixner AM, Verdone BM, Otte CG, Anderson EN, Ramesh N, Shapiro OR, Gale JR, Mauna JC, Mann JR, Copley KE, Daley EL, Ortega JA, Cicardi ME, Kiskinis E, Kofler J, Pandey UB, Trotti D, Donnelly CJ
Nature communications 2022 Jun 13;13(1):3380
Nature communications 2022 Jun 13;13(1):3380
Sequestration of TDP-43(216-414) Aggregates by Cytoplasmic Expression of the proSAAS Chaperone.
Peinado JR, Chaplot K, Jarvela TS, Barbieri EM, Shorter J, Lindberg I
ACS chemical neuroscience 2022 Jun 1;13(11):1651-1665
ACS chemical neuroscience 2022 Jun 1;13(11):1651-1665
Prolyl Isomerase Pin1 Expression in the Spinal Motor Neurons of Patients With Sporadic Amyotrophic Lateral Sclerosis.
Kato H, Naito M, Saito T, Hideyama T, Suzuki Y, Kimura T, Kwak S, Aizawa H
Journal of clinical neurology (Seoul, Korea) 2022 Jul;18(4):463-469
Journal of clinical neurology (Seoul, Korea) 2022 Jul;18(4):463-469
A quantitative biology approach correlates neuronal toxicity with the largest inclusions of TDP-43.
Cascella R, Bigi A, Riffert DG, Gagliani MC, Ermini E, Moretti M, Cortese K, Cecchi C, Chiti F
Science advances 2022 Jul 29;8(30):eabm6376
Science advances 2022 Jul 29;8(30):eabm6376
Nuclear RNA binding regulates TDP-43 nuclear localization and passive nuclear export.
Duan L, Zaepfel BL, Aksenova V, Dasso M, Rothstein JD, Kalab P, Hayes LR
Cell reports 2022 Jul 19;40(3):111106
Cell reports 2022 Jul 19;40(3):111106
Hyperosmotic-stress-induced liquid-liquid phase separation of ALS-related proteins in the nucleus.
Gao C, Gu J, Zhang H, Jiang K, Tang L, Liu R, Zhang L, Zhang P, Liu C, Dai B, Song J
Cell reports 2022 Jul 19;40(3):111086
Cell reports 2022 Jul 19;40(3):111086
TREM2 interacts with TDP-43 and mediates microglial neuroprotection against TDP-43-related neurodegeneration.
Xie M, Liu YU, Zhao S, Zhang L, Bosco DB, Pang YP, Zhong J, Sheth U, Martens YA, Zhao N, Liu CC, Zhuang Y, Wang L, Dickson DW, Mattson MP, Bu G, Wu LJ
Nature neuroscience 2022 Jan;25(1):26-38
Nature neuroscience 2022 Jan;25(1):26-38
Chemically induced senescence in human stem cell-derived neurons promotes phenotypic presentation of neurodegeneration.
Fathi A, Mathivanan S, Kong L, Petersen AJ, Harder CRK, Block J, Miller JM, Bhattacharyya A, Wang D, Zhang SC
Aging cell 2022 Jan;21(1):e13541
Aging cell 2022 Jan;21(1):e13541
miR-23a suppression accelerates functional decline in the rNLS8 mouse model of TDP-43 proteinopathy.
Tsitkanou S, Della Gatta PA, Abbott G, Wallace MA, Lindsay A, Gerlinger-Romero F, Walker AK, Foletta VC, Russell AP
Neurobiology of disease 2022 Jan;162:105559
Neurobiology of disease 2022 Jan;162:105559
Analysis of the Biomarkers for Neurodegenerative Diseases in Aged Progranulin Deficient Mice.
Zhao X, Hasan S, Liou B, Lin Y, Sun Y, Liu C
International journal of molecular sciences 2022 Jan 6;23(2)
International journal of molecular sciences 2022 Jan 6;23(2)
Developmental deficits and staging of dynamics of age associated Alzheimer's disease neurodegeneration and neuronal loss in subjects with Down syndrome.
Wegiel J, Flory M, Kuchna I, Nowicki K, Wegiel J, Ma SY, Zhong N, Bobrowicz TW, de Leon M, Lai F, Silverman WP, Wisniewski T
Acta neuropathologica communications 2022 Jan 4;10(1):2
Acta neuropathologica communications 2022 Jan 4;10(1):2
CHMP2B regulates TDP-43 phosphorylation and cytotoxicity independent of autophagy via CK1.
Deng X, Sun X, Yue W, Duan Y, Hu R, Zhang K, Ni J, Cui J, Wang Q, Chen Y, Li A, Fang Y
The Journal of cell biology 2022 Jan 3;221(1)
The Journal of cell biology 2022 Jan 3;221(1)
Structure-Based Analysis of Protein Cluster Size for Super-Resolution Microscopy in the Nervous System.
Wong CE, Chu YP, Tsai KJ
Biomedicines 2022 Jan 27;10(2)
Biomedicines 2022 Jan 27;10(2)
Feeder-supported in vitro exercise model using human satellite cells from patients with sporadic inclusion body myositis.
Li Y, Chen W, Ogawa K, Koide M, Takahashi T, Hagiwara Y, Itoi E, Aizawa T, Tsuchiya M, Izumi R, Suzuki N, Aoki M, Kanzaki M
Scientific reports 2022 Jan 20;12(1):1082
Scientific reports 2022 Jan 20;12(1):1082
Loss of TDP-43 function and rimmed vacuoles persist after T cell depletion in a xenograft model of sporadic inclusion body myositis.
Britson KA, Ling JP, Braunstein KE, Montagne JM, Kastenschmidt JM, Wilson A, Ikenaga C, Tsao W, Pinal-Fernandez I, Russell KA, Reed N, Mozaffar T, Wagner KR, Ostrow LW, Corse AM, Mammen AL, Villalta SA, Larman HB, Wong PC, Lloyd TE
Science translational medicine 2022 Jan 19;14(628):eabi9196
Science translational medicine 2022 Jan 19;14(628):eabi9196
Sublethal enteroviral infection exacerbates disease progression in an ALS mouse model.
Xue YC, Liu H, Mohamud Y, Bahreyni A, Zhang J, Cashman NR, Luo H
Journal of neuroinflammation 2022 Jan 12;19(1):16
Journal of neuroinflammation 2022 Jan 12;19(1):16
Effects of intracellular calcium accumulation on proteins encoded by the major genes underlying amyotrophic lateral sclerosis.
De Marco G, Lomartire A, Manera U, Canosa A, Grassano M, Casale F, Fuda G, Salamone P, Rinaudo MT, Colombatto S, Moglia C, Chiò A, Calvo A
Scientific reports 2022 Jan 10;12(1):395
Scientific reports 2022 Jan 10;12(1):395
Vulnerable exons, like ACADM exon 5, are highly dependent on maintaining a correct balance between splicing enhancers and silencers.
Holm LL, Doktor TK, Hansen MB, Petersen USS, Andresen BS
Human mutation 2022 Feb;43(2):253-265
Human mutation 2022 Feb;43(2):253-265
TDP-43 Pathology and Prionic Behavior in Human Cellular Models of Alzheimer's Disease Patients.
Cuevas EP, Rodríguez-Fernández A, Palomo V, Martínez A, Martín-Requero Á
Biomedicines 2022 Feb 5;10(2)
Biomedicines 2022 Feb 5;10(2)
A postzygotic de novo NCDN mutation identified in a sporadic FTLD patient results in neurochondrin haploinsufficiency and altered FUS granule dynamics.
Nicolas G, Sévigny M, Lecoquierre F, Marguet F, Deschênes A, Del Pelaez MC, Feuillette S, Audebrand A, Lecourtois M, Rousseau S, Richard AC, Cassinari K, Deramecourt V, Duyckaerts C, Boland A, Deleuze JF, Meyer V, Clarimon Echavarria J, Gelpi E, Akiyama H, Hasegawa M, Kawakami I, Wong TH, Van Rooij JGJ, Van Swieten JC, Campion D, Dutchak PA, Wallon D, Lavoie-Cardinal F, Laquerrière A, Rovelet-Lecrux A, Sephton CF
Acta neuropathologica communications 2022 Feb 12;10(1):20
Acta neuropathologica communications 2022 Feb 12;10(1):20
Association of Neurofibrillary Tangle Distribution With Age at Onset-Related Clinical Heterogeneity in Alzheimer Disease: An Autopsy Study.
Smirnov DS, Salmon DP, Galasko D, Goodwill VS, Hansen LA, Zhao Y, Edland SD, Léger GC, Peavy GM, Jacobs DM, Rissman R, Pizzo DP, Hiniker A
Neurology 2022 Feb 1;98(5):e506-e517
Neurology 2022 Feb 1;98(5):e506-e517
Sigma-1 receptor agonist PRE-084 confers protection against TAR DNA-binding protein-43 toxicity through NRF2 signalling.
Lasbleiz C, Peyrel A, Tarot P, Sarniguet J, Crouzier L, Cubedo N, Delprat B, Rossel M, Maurice T, Liévens JC
Redox biology 2022 Dec;58:102542
Redox biology 2022 Dec;58:102542
TDP-43 condensates and lipid droplets regulate the reactivity of microglia and regeneration after traumatic brain injury.
Zambusi A, Novoselc KT, Hutten S, Kalpazidou S, Koupourtidou C, Schieweck R, Aschenbroich S, Silva L, Yazgili AS, van Bebber F, Schmid B, Möller G, Tritscher C, Stigloher C, Delbridge C, Sirko S, Günes ZI, Liebscher S, Schlegel J, Aliee H, Theis F, Meiners S, Kiebler M, Dormann D, Ninkovic J
Nature neuroscience 2022 Dec;25(12):1608-1625
Nature neuroscience 2022 Dec;25(12):1608-1625
Diverse limbic comorbidities cause limbic and temporal atrophy in lewy body disease.
Sakurai K, Kaneda D, Morimoto S, Uchida Y, Inui S, Kimura Y, Cai C, Kato T, Ito K, Hashizume Y
Parkinsonism & related disorders 2022 Dec;105:52-57
Parkinsonism & related disorders 2022 Dec;105:52-57
Therapeutic Effect of Rapamycin on TDP-43-Related Pathogenesis in Ischemic Stroke.
Tsou YS, Lai JH, Chen KY, Chang CF, Huang CC
International journal of molecular sciences 2022 Dec 30;24(1)
International journal of molecular sciences 2022 Dec 30;24(1)
An autopsy case of amyotrophic lateral sclerosis with striatonigral and pallidoluysian degeneration and cat's-eye-shaped neuronal nuclear inclusions.
Kon T, Mori F, Kinoshita I, Nakamura T, Nishijima H, Suzuki C, Goto S, Kijima H, Tomiyama M, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2022 Aug;42(4):329-338
Neuropathology : official journal of the Japanese Society of Neuropathology 2022 Aug;42(4):329-338
Unraveling the toxic effects mediated by the neurodegenerative disease-associated S375G mutation of TDP-43 and its S375E phosphomimetic variant.
Paron F, Barattucci S, Cappelli S, Romano M, Berlingieri C, Stuani C, Laurents D, Mompeán M, Buratti E
The Journal of biological chemistry 2022 Aug;298(8):102252
The Journal of biological chemistry 2022 Aug;298(8):102252
Aberrant neural activity in prefrontal pyramidal neurons lacking TDP-43 precedes neuron loss.
Liang B, Thapa R, Zhang G, Moffitt C, Zhang Y, Zhang L, Johnston A, Ruby HP, Barbera G, Wong PC, Zhang Z, Chen R, Lin DT, Li Y
Progress in neurobiology 2022 Aug;215:102297
Progress in neurobiology 2022 Aug;215:102297
ALS-linked KIF5A ΔExon27 mutant causes neuronal toxicity through gain-of-function.
Pant DC, Parameswaran J, Rao L, Loss I, Chilukuri G, Parlato R, Shi L, Glass JD, Bassell GJ, Koch P, Yilmaz R, Weishaupt JH, Gennerich A, Jiang J
EMBO reports 2022 Aug 3;23(8):e54234
EMBO reports 2022 Aug 3;23(8):e54234
A motor neuron disease mouse model reveals a non-canonical profile of senescence biomarkers.
Torres P, Anerillas C, Ramírez-Núñez O, Fernàndez A, Encinas M, Povedano M, Andrés-Benito P, Ferrer I, Ayala V, Pamplona R, Portero-Otín M
Disease models & mechanisms 2022 Aug 1;15(8)
Disease models & mechanisms 2022 Aug 1;15(8)
Testing of the therapeutic efficacy and safety of AMPA receptor RNA aptamers in an ALS mouse model.
Akamatsu M, Yamashita T, Teramoto S, Huang Z, Lynch J, Toda T, Niu L, Kwak S
Life science alliance 2022 Apr;5(4)
Life science alliance 2022 Apr;5(4)
Long-standing multiple system atrophy-Parkinsonism with limbic and FTLD-type α-synuclein pathology.
Coughlin DG, Dryden I, Goodwill VS, Pizzo DP, Wright B, Lessig S, Galasko D, MacKenzie IR, Hiniker A
Neuropathology and applied neurobiology 2022 Apr;48(3):e12783
Neuropathology and applied neurobiology 2022 Apr;48(3):e12783
Plasma biomarkers for Alzheimer's Disease in relation to neuropathology and cognitive change.
Smirnov DS, Ashton NJ, Blennow K, Zetterberg H, Simrén J, Lantero-Rodriguez J, Karikari TK, Hiniker A, Rissman RA, Salmon DP, Galasko D
Acta neuropathologica 2022 Apr;143(4):487-503
Acta neuropathologica 2022 Apr;143(4):487-503
Heterozygous frameshift variants in HNRNPA2B1 cause early-onset oculopharyngeal muscular dystrophy.
Kim HJ, Mohassel P, Donkervoort S, Guo L, O'Donovan K, Coughlin M, Lornage X, Foulds N, Hammans SR, Foley AR, Fare CM, Ford AF, Ogasawara M, Sato A, Iida A, Munot P, Ambegaonkar G, Phadke R, O'Donovan DG, Buchert R, Grimmel M, Töpf A, Zaharieva IT, Brady L, Hu Y, Lloyd TE, Klein A, Steinlin M, Kuster A, Mercier S, Marcorelles P, Péréon Y, Fleurence E, Manzur A, Ennis S, Upstill-Goddard R, Bello L, Bertolin C, Pegoraro E, Salviati L, French CE, Shatillo A, Raymond FL, Haack TB, Quijano-Roy S, Böhm J, Nelson I, Stojkovic T, Evangelista T, Straub V, Romero NB, Laporte J, Muntoni F, Nishino I, Tarnopolsky MA, Shorter J, Bönnemann CG, Taylor JP
Nature communications 2022 Apr 28;13(1):2306
Nature communications 2022 Apr 28;13(1):2306
Disease-linked TDP-43 hyperphosphorylation suppresses TDP-43 condensation and aggregation.
Gruijs da Silva LA, Simonetti F, Hutten S, Riemenschneider H, Sternburg EL, Pietrek LM, Gebel J, Dötsch V, Edbauer D, Hummer G, Stelzl LS, Dormann D
The EMBO journal 2022 Apr 19;41(8):e108443
The EMBO journal 2022 Apr 19;41(8):e108443
Homotypic fibrillization of TMEM106B across diverse neurodegenerative diseases.
Chang A, Xiang X, Wang J, Lee C, Arakhamia T, Simjanoska M, Wang C, Carlomagno Y, Zhang G, Dhingra S, Thierry M, Perneel J, Heeman B, Forgrave LM, DeTure M, DeMarco ML, Cook CN, Rademakers R, Dickson DW, Petrucelli L, Stowell MHB, Mackenzie IRA, Fitzpatrick AWP
Cell 2022 Apr 14;185(8):1346-1355.e15
Cell 2022 Apr 14;185(8):1346-1355.e15
Physiological tissue-specific and age-related reduction of mouse TDP-43 levels is regulated by epigenetic modifications.
Pacetti M, De Conti L, Marasco LE, Romano M, Rashid MM, Nubiè M, Baralle FE, Baralle M
Disease models & mechanisms 2022 Apr 1;15(4)
Disease models & mechanisms 2022 Apr 1;15(4)
Polyadenylated RNA and RNA-Binding Proteins Exhibit Unique Response to Hyperosmotic Stress.
Zaepfel BL, Rothstein JD
Frontiers in cell and developmental biology 2021;9:809859
Frontiers in cell and developmental biology 2021;9:809859
Neuronal VPS35 deletion induces spinal cord motor neuron degeneration and early post-natal lethality.
Sargent D, Cunningham LA, Dues DJ, Ma Y, Kordich JJ, Mercado G, Brundin P, Cowell RM, Moore DJ
Brain communications 2021;3(3):fcab208
Brain communications 2021;3(3):fcab208
Behavioral Deficits in Mice with Postnatal Disruption of Ndel1 in Forebrain Excitatory Neurons: Implications for Epilepsy and Neuropsychiatric Disorders.
Gavrilovici C, Jiang Y, Kiroski I, Sterley TL, Vandal M, Bains J, Park SK, Rho JM, Teskey GC, Nguyen MD
Cerebral cortex communications 2021;2(1):tgaa096
Cerebral cortex communications 2021;2(1):tgaa096
hnRNP A1B, a Splice Variant of HNRNPA1, Is Spatially and Temporally Regulated.
Gagné M, Deshaies JE, Sidibé H, Benchaar Y, Arbour D, Dubinski A, Litt G, Peyrard S, Robitaille R, Sephton CF, Vande Velde C
Frontiers in neuroscience 2021;15:724307
Frontiers in neuroscience 2021;15:724307
Repeated Low-Level Blast Acutely Alters Brain Cytokines, Neurovascular Proteins, Mechanotransduction, and Neurodegenerative Markers in a Rat Model.
Heyburn L, Abutarboush R, Goodrich S, Urioste R, Batuure A, Wheel J, Wilder DM, Arun P, Ahlers ST, Long JB, Sajja VS
Frontiers in cellular neuroscience 2021;15:636707
Frontiers in cellular neuroscience 2021;15:636707
Targeted Quantification of Detergent-Insoluble RNA-Binding Proteins in Human Brain Reveals Stage and Disease Specific Co-aggregation in Alzheimer's Disease.
Guo Q, Dammer EB, Zhou M, Kundinger SR, Gearing M, Lah JJ, Levey AI, Shulman JM, Seyfried NT
Frontiers in molecular neuroscience 2021;14:623659
Frontiers in molecular neuroscience 2021;14:623659
TDP-43 proteinopathy impairs mRNP granule mediated postsynaptic translation and mRNA metabolism.
Wong CE, Jin LW, Chu YP, Wei WY, Ho PC, Tsai KJ
Theranostics 2021;11(1):330-345
Theranostics 2021;11(1):330-345
Pathological manifestation of human endogenous retrovirus K in frontotemporal dementia.
Phan K, He Y, Fu Y, Dzamko N, Bhatia S, Gold J, Rowe D, Ke YD, Ittner LM, Hodges JR, Piguet O, Kiernan MC, Halliday GM, Kim WS
Communications medicine 2021;1
Communications medicine 2021;1
Impact of a frequent nearsplice SOD1 variant in amyotrophic lateral sclerosis: optimising SOD1 genetic screening for gene therapy opportunities.
Muratet F, Teyssou E, Chiot A, Boillée S, Lobsiger CS, Bohl D, Gyorgy B, Guegan J, Marie Y, Amador MDM, Salachas F, Meininger V, Bernard E, Antoine JC, Camdessanché JP, Camu W, Cazeneuve C, Fauret-Amsellem AL, Leguern E, Mouzat K, Guissart C, Lumbroso S, Corcia P, Vourc'h P, Grapperon AM, Attarian S, Verschueren A, Seilhean D, Millecamps S
Journal of neurology, neurosurgery, and psychiatry 2021 Sep;92(9):942-949
Journal of neurology, neurosurgery, and psychiatry 2021 Sep;92(9):942-949
Riluzole does not ameliorate disease caused by cytoplasmic TDP-43 in a mouse model of amyotrophic lateral sclerosis.
Wright AL, Della Gatta PA, Le S, Berning BA, Mehta P, Jacobs KR, Gul H, San Gil R, Hedl TJ, Riddell WR, Watson O, Keating SS, Venturato J, Chung RS, Atkin JD, Lee A, Shi B, Blizzard CA, Morsch M, Walker AK
The European journal of neuroscience 2021 Sep;54(6):6237-6255
The European journal of neuroscience 2021 Sep;54(6):6237-6255
ATG14 and RB1CC1 play essential roles in maintaining muscle homeostasis.
Li D, Vogel P, Li-Harms X, Wang B, Kundu M
Autophagy 2021 Sep;17(9):2576-2585
Autophagy 2021 Sep;17(9):2576-2585
TDP-43 mediates SREBF2-regulated gene expression required for oligodendrocyte myelination.
Ho WY, Chang JC, Lim K, Cazenave-Gassiot A, Nguyen AT, Foo JC, Muralidharan S, Viera-Ortiz A, Ong SJM, Hor JH, Agrawal I, Hoon S, Arogundade OA, Rodriguez MJ, Lim SM, Kim SH, Ravits J, Ng SY, Wenk MR, Lee EB, Tucker-Kellogg G, Ling SC
The Journal of cell biology 2021 Sep 6;220(9)
The Journal of cell biology 2021 Sep 6;220(9)
MATR3 F115C knock-in mice do not exhibit motor defects or neuropathological features of ALS.
van Bruggen R, Maksimovic K, You J, Tran DD, Lee HJ, Khan M, Kao CS, Kim JR, Cho W, Chen XXL, Park J
Biochemical and biophysical research communications 2021 Sep 3;568:48-54
Biochemical and biophysical research communications 2021 Sep 3;568:48-54
TDP-43 condensation properties specify its RNA-binding and regulatory repertoire.
Hallegger M, Chakrabarti AM, Lee FCY, Lee BL, Amalietti AG, Odeh HM, Copley KE, Rubien JD, Portz B, Kuret K, Huppertz I, Rau F, Patani R, Fawzi NL, Shorter J, Luscombe NM, Ule J
Cell 2021 Sep 2;184(18):4680-4696.e22
Cell 2021 Sep 2;184(18):4680-4696.e22
A microfluidic approach to rescue ALS motor neuron degeneration using rapamycin.
Chennampally P, Sayed-Zahid A, Soundararajan P, Sharp J, Cox GA, Collins SD, Smith RL
Scientific reports 2021 Sep 13;11(1):18168
Scientific reports 2021 Sep 13;11(1):18168
Fast Progression in Amyotrophic Lateral Sclerosis Is Associated With Greater TDP-43 Burden in Spinal Cord.
Cathcart SJ, Appel SH, Peterson LE, Greene EP, Powell SZ, Arumanayagam AS, Rivera AL, Cykowski MD
Journal of neuropathology and experimental neurology 2021 Sep 10;80(8):754-763
Journal of neuropathology and experimental neurology 2021 Sep 10;80(8):754-763
TMEM106B modifies TDP-43 pathology in human ALS brain and cell-based models of TDP-43 proteinopathy.
Mao F, Robinson JL, Unger T, Posavi M, Amado DA, Elman L, Grossman M, Wolk DA, Lee EB, Van Deerlin VM, Porta S, Lee VMY, Trojanowski JQ, Chen-Plotkin AS
Acta neuropathologica 2021 Oct;142(4):629-642
Acta neuropathologica 2021 Oct;142(4):629-642
Establishment of chemically oligomerizable TAR DNA-binding protein-43 which mimics amyotrophic lateral sclerosis pathology in mammalian cells.
Yamanaka Y, Miyagi T, Harada Y, Kuroda M, Kanekura K
Laboratory investigation; a journal of technical methods and pathology 2021 Oct;101(10):1331-1340
Laboratory investigation; a journal of technical methods and pathology 2021 Oct;101(10):1331-1340
Ribonuclease recruitment using a small molecule reduced c9ALS/FTD r(G(4)C(2)) repeat expansion in vitro and in vivo ALS models.
Bush JA, Aikawa H, Fuerst R, Li Y, Ursu A, Meyer SM, Benhamou RI, Chen JL, Khan T, Wagner-Griffin S, Van Meter MJ, Tong Y, Olafson H, McKee KK, Childs-Disney JL, Gendron TF, Zhang Y, Coyne AN, Wang ET, Yildirim I, Wang KW, Petrucelli L, Rothstein JD, Disney MD
Science translational medicine 2021 Oct 27;13(617):eabd5991
Science translational medicine 2021 Oct 27;13(617):eabd5991
Transmission of amyloid-beta and tau pathologies is associated with cognitive impairments in a primate.
Lam S, Petit F, Hérard AS, Boluda S, Eddarkaoui S, Guillermier M, Brain Bank Neuro-C. E. B. Neuropathology Network, Buée L, Duyckaerts C, Haïk S, Picq JL, Dhenain M
Acta neuropathologica communications 2021 Oct 12;9(1):165
Acta neuropathologica communications 2021 Oct 12;9(1):165
FTLD Patient-Derived Fibroblasts Show Defective Mitochondrial Function and Accumulation of p62.
Leskelä S, Hoffmann D, Rostalski H, Huber N, Wittrahm R, Hartikainen P, Korhonen V, Leinonen V, Hiltunen M, Solje E, Remes AM, Haapasalo A
Molecular neurobiology 2021 Nov;58(11):5438-5458
Molecular neurobiology 2021 Nov;58(11):5438-5458
Phosphorylation regulates arginine-rich RNA-binding protein solubility and oligomerization.
Kundinger SR, Dammer EB, Yin L, Hurst C, Shapley S, Ping L, Khoshnevis S, Ghalei H, Duong DM, Seyfried NT
The Journal of biological chemistry 2021 Nov;297(5):101306
The Journal of biological chemistry 2021 Nov;297(5):101306
Long non-coding RNA NEAT1_1 ameliorates TDP-43 toxicity in in vivo models of TDP-43 proteinopathy.
Matsukawa K, Kukharsky MS, Park SK, Park S, Watanabe N, Iwatsubo T, Hashimoto T, Liebman SW, Shelkovnikova TA
RNA biology 2021 Nov;18(11):1546-1554
RNA biology 2021 Nov;18(11):1546-1554
Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins.
Altman T, Ionescu A, Ibraheem A, Priesmann D, Gradus-Pery T, Farberov L, Alexandra G, Shelestovich N, Dafinca R, Shomron N, Rage F, Talbot K, Ward ME, Dori A, Krüger M, Perlson E
Nature communications 2021 Nov 25;12(1):6914
Nature communications 2021 Nov 25;12(1):6914
Mid to late-life scores of depression in the cognitively healthy are associated with cognitive status and Alzheimer's disease pathology at death.
Robinson AC, Roncaroli F, Davidson YS, Minshull J, Heal C, Montaldi D, Payton A, Horan MA, Pendleton N, Mann DMA
International journal of geriatric psychiatry 2021 May;36(5):713-721
International journal of geriatric psychiatry 2021 May;36(5):713-721
Mislocalisation of TDP-43 to the cytoplasm causes cortical hyperexcitability and reduced excitatory neurotransmission in the motor cortex.
Dyer MS, Reale LA, Lewis KE, Walker AK, Dickson TC, Woodhouse A, Blizzard CA
Journal of neurochemistry 2021 May;157(4):1300-1315
Journal of neurochemistry 2021 May;157(4):1300-1315
Globular glial tauopathy with a mutation in MAPT and unusual TDP-43 proteinopathy in a patient with behavioural-variant frontotemporal dementia.
Forrest SL, Shepherd CE, McCann H, Kwok JB, Halliday GM, Kril JJ
Acta neuropathologica 2021 May;141(5):791-794
Acta neuropathologica 2021 May;141(5):791-794
Patterns of neuronal Rhes as a novel hallmark of tauopathies.
Ehrenberg AJ, Leng K, Letourneau KN, Hernandez I, Lew C, Seeley WW, Spina S, Miller B, Heinsen H, Kampmann M, Kosik KS, Grinberg LT
Acta neuropathologica 2021 May;141(5):651-666
Acta neuropathologica 2021 May;141(5):651-666
Traumatic injury compromises nucleocytoplasmic transport and leads to TDP-43 pathology.
Anderson EN, Morera AA, Kour S, Cherry JD, Ramesh N, Gleixner A, Schwartz JC, Ebmeier C, Old W, Donnelly CJ, Cheng JP, Kline AE, Kofler J, Stein TD, Pandey UB
eLife 2021 May 26;10
eLife 2021 May 26;10
FUS/TLS Suppresses Enterovirus Replication and Promotes Antiviral Innate Immune Responses.
Xue YC, Ng CS, Mohamud Y, Fung G, Liu H, Bahreyni A, Zhang J, Luo H
Journal of virology 2021 May 24;95(12)
Journal of virology 2021 May 24;95(12)
Characterization of the human TARDBP gene promoter.
Baralle M, Romano M
Scientific reports 2021 May 17;11(1):10438
Scientific reports 2021 May 17;11(1):10438
Genetic screening of ANXA11 revealed novel mutations linked to amyotrophic lateral sclerosis.
Teyssou E, Muratet F, Amador MD, Ferrien M, Lautrette G, Machat S, Boillée S, Larmonier T, Saker S, Leguern E, Cazeneuve C, Marie Y, Guegan J, Gyorgy B, Cintas P, Meininger V, Le Forestier N, Salachas F, Couratier P, Camu W, Seilhean D, Millecamps S
Neurobiology of aging 2021 Mar;99:102.e11-102.e20
Neurobiology of aging 2021 Mar;99:102.e11-102.e20
Identification of SRSF10 as a regulator of SMN2 ISS-N1.
Frederiksen SB, Holm LL, Larsen MR, Doktor TK, Andersen HS, Hastings ML, Hua Y, Krainer AR, Andresen BS
Human mutation 2021 Mar;42(3):246-260
Human mutation 2021 Mar;42(3):246-260
A novel temporal-predominant neuro-astroglial tauopathy associated with TMEM106B gene polymorphism in FTLD/ALS-TDP.
Llibre-Guerra JJ, Lee SE, Suemoto CK, Ehrenberg AJ, Kovacs GG, Karydas A, Staffaroni A, Franca Resende EP, Kim EJ, Hwang JH, Ramos EM, Wojta KJ, Pasquini L, Pang SY, Spina S, Allen IE, Kramer J, Miller BL, Seeley WW, Grinberg LT
Brain pathology (Zurich, Switzerland) 2021 Mar;31(2):267-282
Brain pathology (Zurich, Switzerland) 2021 Mar;31(2):267-282
Pontine Arteriolosclerosis and Locus Coeruleus Oxidative Stress Differentiate Resilience from Mild Cognitive Impairment in a Clinical Pathologic Cohort.
Kelly SC, Nelson PT, Counts SE
Journal of neuropathology and experimental neurology 2021 Mar 22;80(4):325-335
Journal of neuropathology and experimental neurology 2021 Mar 22;80(4):325-335
Hippocampal Sclerosis in Frontotemporal Dementia: When Vascular Pathology Meets Neurodegeneration.
Sieben A, Van Langenhove T, Vermeiren Y, Gossye H, Praet M, Vanhauwaert D, Cousaert C, Engelborghs S, Raedt R, Boon P, Santens P, De Deyn PP, Bracke KR, De Meulemeester K, Van Broeckhoven C, Martin JJ, Bjerke M
Journal of neuropathology and experimental neurology 2021 Mar 22;80(4):313-324
Journal of neuropathology and experimental neurology 2021 Mar 22;80(4):313-324
Identification of Genetic Modifiers of TDP-43: Inflammatory Activation of Astrocytes for Neuroinflammation.
Kim JH, Rahman MH, Park D, Jo M, Kim HJ, Suk K
Cells 2021 Mar 18;10(3)
Cells 2021 Mar 18;10(3)
The E50K optineurin mutation impacts autophagy-mediated degradation of TDP-43 and leads to RGC apoptosis in vivo and in vitro.
Zhang S, Shao Z, Liu X, Hou M, Cheng F, Lei D, Yuan H
Cell death discovery 2021 Mar 15;7(1):49
Cell death discovery 2021 Mar 15;7(1):49
Multiple Sclerosis-Associated hnRNPA1 Mutations Alter hnRNPA1 Dynamics and Influence Stress Granule Formation.
Clarke JWE, Thibault PA, Salapa HE, Kim DE, Hutchinson C, Levin MC
International journal of molecular sciences 2021 Mar 12;22(6)
International journal of molecular sciences 2021 Mar 12;22(6)
Deferoxamine mesylate improves splicing and GAA activity of the common c.-32-13T>G allele in late-onset PD patient fibroblasts.
Buratti E, Peruzzo P, Braga L, Zanin I, Stuani C, Goina E, Romano M, Giacca M, Dardis A
Molecular therapy. Methods & clinical development 2021 Mar 12;20:227-236
Molecular therapy. Methods & clinical development 2021 Mar 12;20:227-236
TDP-43 maximizes nerve conduction velocity by repressing a cryptic exon for paranodal junction assembly in Schwann cells.
Chang KJ, Agrawal I, Vainshtein A, Ho WY, Xin W, Tucker-Kellogg G, Susuki K, Peles E, Ling SC, Chan JR
eLife 2021 Mar 10;10
eLife 2021 Mar 10;10
Staufen1 in Human Neurodegeneration.
Paul S, Dansithong W, Figueroa KP, Gandelman M, Scoles DR, Pulst SM
Annals of neurology 2021 Jun;89(6):1114-1128
Annals of neurology 2021 Jun;89(6):1114-1128
MIR-NATs repress MAPT translation and aid proteostasis in neurodegeneration.
Simone R, Javad F, Emmett W, Wilkins OG, Almeida FL, Barahona-Torres N, Zareba-Paslawska J, Ehteramyan M, Zuccotti P, Modelska A, Siva K, Virdi GS, Mitchell JS, Harley J, Kay VA, Hondhamuni G, Trabzuni D, Ryten M, Wray S, Preza E, Kia DA, Pittman A, Ferrari R, Manzoni C, Lees A, Hardy JA, Denti MA, Quattrone A, Patani R, Svenningsson P, Warner TT, Plagnol V, Ule J, de Silva R
Nature 2021 Jun;594(7861):117-123
Nature 2021 Jun;594(7861):117-123
Expression of C9orf72 hexanucleotide repeat expansion leads to formation of RNA foci and dipeptide repeat proteins but does not influence autophagy or proteasomal function in neuronal cells.
Leskelä S, Huber N, Hoffmann D, Rostalski H, Remes AM, Takalo M, Hiltunen M, Haapasalo A
Biochimica et biophysica acta. Molecular cell research 2021 Jun;1868(7):119021
Biochimica et biophysica acta. Molecular cell research 2021 Jun;1868(7):119021
O-GlcNAcylation of TDP-43 suppresses proteinopathies and promotes TDP-43's mRNA splicing activity.
Zhao MJ, Yao X, Wei P, Zhao C, Cheng M, Zhang D, Xue W, He WT, Xue W, Zuo X, Jiang LL, Luo Z, Song J, Shu WJ, Yuan HY, Liang Y, Sun H, Zhou Y, Zhou Y, Zheng L, Hu HY, Wang J, Du HN
EMBO reports 2021 Jun 4;22(6):e51649
EMBO reports 2021 Jun 4;22(6):e51649
Mouse closed head traumatic brain injury replicates the histological tau pathology pattern of human disease: characterization of a novel model and systematic review of the literature.
Kahriman A, Bouley J, Smith TW, Bosco DA, Woerman AL, Henninger N
Acta neuropathologica communications 2021 Jun 29;9(1):118
Acta neuropathologica communications 2021 Jun 29;9(1):118
UBQLN2-HSP70 axis reduces poly-Gly-Ala aggregates and alleviates behavioral defects in the C9ORF72 animal model.
Zhang K, Wang A, Zhong K, Qi S, Wei C, Shu X, Tu WY, Xu W, Xia C, Xiao Y, Chen A, Bai L, Zhang J, Luo B, Wang W, Shen C
Neuron 2021 Jun 16;109(12):1949-1962.e6
Neuron 2021 Jun 16;109(12):1949-1962.e6
Identification of hub molecules of FUS-ALS by Bayesian gene regulatory network analysis of iPSC model: iBRN.
Nogami M, Ishikawa M, Doi A, Sano O, Sone T, Akiyama T, Aoki M, Nakanishi A, Ogi K, Yano M, Okano H
Neurobiology of disease 2021 Jul;155:105364
Neurobiology of disease 2021 Jul;155:105364
Stimulation of mTOR-independent autophagy and mitophagy by rilmenidine exacerbates the phenotype of transgenic TDP-43 mice.
Perera ND, Tomas D, Wanniarachchillage N, Cuic B, Luikinga SJ, Rytova V, Turner BJ
Neurobiology of disease 2021 Jul;154:105359
Neurobiology of disease 2021 Jul;154:105359
Effects of TDP-43 overexpression on neuron proteome and morphology in vitro.
Atkinson RAK, Fair HL, Wilson R, Vickers JC, King AE
Molecular and cellular neurosciences 2021 Jul;114:103627
Molecular and cellular neurosciences 2021 Jul;114:103627
Rapid in vitro quantification of TDP-43 and FUS mislocalisation for screening of gene variants implicated in frontotemporal dementia and amyotrophic lateral sclerosis.
Oyston LJ, Ubiparipovic S, Fitzpatrick L, Hallupp M, Boccanfuso LM, Kwok JB, Dobson-Stone C
Scientific reports 2021 Jul 21;11(1):14881
Scientific reports 2021 Jul 21;11(1):14881
Neuronal VCP loss of function recapitulates FTLD-TDP pathology.
Wani A, Zhu J, Ulrich JD, Eteleeb A, Sauerbeck AD, Reitz SJ, Arhzaouy K, Ikenaga C, Yuede CM, Pittman SK, Wang F, Li S, Benitez BA, Cruchaga C, Kummer TT, Harari O, Chou TF, Schröder R, Clemen CS, Weihl CC
Cell reports 2021 Jul 20;36(3):109399
Cell reports 2021 Jul 20;36(3):109399
CRISPR/Cas9-engineered Drosophila knock-in models to study VCP diseases.
Wall JM, Basu A, Zunica ERM, Dubuisson OS, Pergola K, Broussard JP, Kirwan JP, Axelrod CL, Johnson AE
Disease models & mechanisms 2021 Jul 1;14(7)
Disease models & mechanisms 2021 Jul 1;14(7)
Hemizygous deletion of Tbk1 worsens neuromuscular junction pathology in TDP-43(G298S) transgenic mice.
Sieverding K, Ulmer J, Bruno C, Satoh T, Tsao W, Freischmidt A, Akira S, Wong PC, Ludolph AC, Danzer KM, Lobsiger CS, Brenner D, Weishaupt JH
Experimental neurology 2021 Jan;335:113496
Experimental neurology 2021 Jan;335:113496
Optineurin defects cause TDP43-pathology with autophagic vacuolar formation.
Kurashige T, Kuramochi M, Ohsawa R, Yamashita Y, Shioi G, Morino H, Kamada M, Ayaki T, Ito H, Sotomaru Y, Maruyama H, Kawakami H
Neurobiology of disease 2021 Jan;148:105215
Neurobiology of disease 2021 Jan;148:105215
Active lifestyle enhances protein expression profile in subjects with Lewy body pathology.
Real CC, Suemoto CK, Binda KH, Grinberg LT, Pasqualucci CA, Jacob W, Ferretti-Rebustini REL, Nitrini R, Leite REP, de Britto LR
Dementia & neuropsychologia 2021 Jan-Mar;15(1):41-50
Dementia & neuropsychologia 2021 Jan-Mar;15(1):41-50
Amyloid β and tau pathology in brains of aged pinniped species (sea lion, seal, and walrus).
Takaichi Y, Chambers JK, Takahashi K, Soeda Y, Koike R, Katsumata E, Kita C, Matsuda F, Haritani M, Takashima A, Nakayama H, Uchida K
Acta neuropathologica communications 2021 Jan 7;9(1):10
Acta neuropathologica communications 2021 Jan 7;9(1):10
The FUS gene is dual-coding with both proteins contributing to FUS-mediated toxicity.
Brunet MA, Jacques JF, Nassari S, Tyzack GE, McGoldrick P, Zinman L, Jean S, Robertson J, Patani R, Roucou X
EMBO reports 2021 Jan 7;22(1):e50640
EMBO reports 2021 Jan 7;22(1):e50640
Novel tankyrase inhibitors suppress TDP-43 aggregate formation.
Tanji K, Mori F, Shirai F, Fukami T, Seimiya H, Utsumi J, Kakita A, Wakabayashi K
Biochemical and biophysical research communications 2021 Jan 22;537:85-92
Biochemical and biophysical research communications 2021 Jan 22;537:85-92
Targeting nuclear protein TDP-43 by cell division cycle kinase 7 inhibitors: A new therapeutic approach for amyotrophic lateral sclerosis.
Rojas-Prats E, Martinez-Gonzalez L, Gonzalo-Consuegra C, Liachko NF, Perez C, Ramírez D, Kraemer BC, Martin-Requero Á, Perez DI, Gil C, de Lago E, Martinez A
European journal of medicinal chemistry 2021 Jan 15;210:112968
European journal of medicinal chemistry 2021 Jan 15;210:112968
Small molecule targeting r(UGGAA)(n) disrupts RNA foci and alleviates disease phenotype in Drosophila model.
Shibata T, Nagano K, Ueyama M, Ninomiya K, Hirose T, Nagai Y, Ishikawa K, Kawai G, Nakatani K
Nature communications 2021 Jan 11;12(1):236
Nature communications 2021 Jan 11;12(1):236
Clinicopathological Staging of Dynamics of Neurodegeneration and Neuronal Loss in Alzheimer Disease.
Wegiel J, Flory M, Kuchna I, Nowicki K, Ma SY, Wegiel J, Badmaev E, Leon M, Wisniewski T, Reisberg B
Journal of neuropathology and experimental neurology 2021 Jan 1;80(1):21-44
Journal of neuropathology and experimental neurology 2021 Jan 1;80(1):21-44
Repetitive Traumatic Brain Injury Is Associated With TDP-43 Alterations, Neurodegeneration, and Glial Activation in Mice.
Rajič Bumber J, Pilipović K, Janković T, Dolenec P, Gržeta N, Križ J, Župan G
Journal of neuropathology and experimental neurology 2021 Jan 1;80(1):2-14
Journal of neuropathology and experimental neurology 2021 Jan 1;80(1):2-14
Multiple system aging-related tau astrogliopathy with complex proteinopathy in an oligosymptomatic octogenarian.
Klotz S, Fischer P, Hinterberger M, Ricken G, Hönigschnabl S, Gelpi E, Kovacs GG
Neuropathology : official journal of the Japanese Society of Neuropathology 2021 Feb;41(1):72-83
Neuropathology : official journal of the Japanese Society of Neuropathology 2021 Feb;41(1):72-83
TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS.
Zuo X, Zhou J, Li Y, Wu K, Chen Z, Luo Z, Zhang X, Liang Y, Esteban MA, Zhou Y, Fu XD
Nature structural & molecular biology 2021 Feb;28(2):132-142
Nature structural & molecular biology 2021 Feb;28(2):132-142
Therapeutic potential of novel Cell Division Cycle Kinase 7 inhibitors on TDP-43-related pathogenesis such as Frontotemporal Lobar Degeneration (FTLD) and amyotrophic lateral sclerosis (ALS).
Vaca G, Martinez-Gonzalez L, Fernandez A, Rojas-Prats E, Porras G, Cuevas EP, Gil C, Martinez A, Martin-Requero Á
Journal of neurochemistry 2021 Feb;156(3):379-390
Journal of neurochemistry 2021 Feb;156(3):379-390
A novel recessive mutation affecting DNAJB6a causes myofibrillar myopathy.
Qian FY, Guo YD, Zu J, Zhang JH, Zheng YM, Abdoulaye IA, Pan ZH, Xie CM, Gao HC, Zhang ZJ
Acta neuropathologica communications 2021 Feb 8;9(1):23
Acta neuropathologica communications 2021 Feb 8;9(1):23
Pathogenic Huntingtin Repeat Expansions in Patients with Frontotemporal Dementia and Amyotrophic Lateral Sclerosis.
Dewan R, Chia R, Ding J, Hickman RA, Stein TD, Abramzon Y, Ahmed S, Sabir MS, Portley MK, Tucci A, Ibáñez K, Shankaracharya FNU, Keagle P, Rossi G, Caroppo P, Tagliavini F, Waldo ML, Johansson PM, Nilsson CF, American Genome Center (TAGC), FALS Sequencing Consortium, Genomics England Research Consortium, International ALS/FTD Genomics Consortium (iAFGC), International FTD Genetics Consortium (IFGC), International LBD Genomics Consortium (iLBDGC), NYGC ALS Consortium, PROSPECT Consortium, Rowe JB, Benussi L, Binetti G, Ghidoni R, Jabbari E, Viollet C, Glass JD, Singleton AB, Silani V, Ross OA, Ryten M, Torkamani A, Tanaka T, Ferrucci L, Resnick SM, Pickering-Brown S, Brady CB, Kowal N, Hardy JA, Van Deerlin V, Vonsattel JP, Harms MB, Morris HR, Ferrari R, Landers JE, Chiò A, Gibbs JR, Dalgard CL, Scholz SW, Traynor BJ
Neuron 2021 Feb 3;109(3):448-460.e4
Neuron 2021 Feb 3;109(3):448-460.e4
The Periodontium Damage Induces Neuronal Cell Death in the Trigeminal Mesencephalic Nucleus and Neurodegeneration in the Trigeminal Motor Nucleus in C57BL/6J Mice.
Dhar A, Kuramoto E, Fukushima M, Iwai H, Yamanaka A, Goto T
Acta histochemica et cytochemica 2021 Feb 25;54(1):11-19
Acta histochemica et cytochemica 2021 Feb 25;54(1):11-19
PCBP2 Is Downregulated in Degenerating Neurons and Rarely Observed in TDP-43-Positive Inclusions in Sporadic Amyotrophic Lateral Sclerosis.
Yoshimura M, Honda H, Sasagasako N, Mori S, Hamasaki H, Suzuki SO, Ishii T, Ninomiya T, Kira JI, Iwaki T
Journal of neuropathology and experimental neurology 2021 Feb 22;80(3):220-228
Journal of neuropathology and experimental neurology 2021 Feb 22;80(3):220-228
18F-AV-1451 positron emission tomography in neuropathological substrates of corticobasal syndrome.
Goodheart AE, Locascio JJ, Samore WR, Collins JA, Brickhouse M, Schultz A, Touroutoglou A, Johnson KA, Frosch MP, Growdon JH, Dickerson BC, Gomperts SN
Brain : a journal of neurology 2021 Feb 12;144(1):266-277
Brain : a journal of neurology 2021 Feb 12;144(1):266-277
TDP-43 proteinopathy occurs independently of autophagic substrate accumulation and underlies nuclear defects in Niemann-Pick C disease.
Liu EA, Mori E, Hamasaki F, Lieberman AP
Neuropathology and applied neurobiology 2021 Dec;47(7):1019-1032
Neuropathology and applied neurobiology 2021 Dec;47(7):1019-1032
Tau-tubulin kinase 1 phosphorylates TDP-43 at disease-relevant sites and exacerbates TDP-43 pathology.
Tian Y, Wang Y, Jablonski AM, Hu Y, Sugam JA, Koglin M, Stachel SJ, Zhou H, Uslaner JM, Parmentier-Batteur S
Neurobiology of disease 2021 Dec;161:105548
Neurobiology of disease 2021 Dec;161:105548
Thioridazine reverts the phenotype in cellular and Drosophila models of amyotrophic lateral sclerosis by enhancing TDP-43 aggregate clearance.
Cragnaz L, Spinelli G, De Conti L, Bureau EA, Brownlees J, Feiguin F, Romano V, Skoko N, Klima R, Kettleborough CA, Baralle FE, Baralle M
Neurobiology of disease 2021 Dec;160:105515
Neurobiology of disease 2021 Dec;160:105515
Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.
Diaz-Garcia S, Ko VI, Vazquez-Sanchez S, Chia R, Arogundade OA, Rodriguez MJ, Traynor BJ, Cleveland D, Ravits J
Acta neuropathologica 2021 Dec;142(6):985-1001
Acta neuropathologica 2021 Dec;142(6):985-1001
NEAT1 is essential for metabolic changes that promote breast cancer growth and metastasis.
Park MK, Zhang L, Min KW, Cho JH, Yeh CC, Moon H, Hormaechea-Agulla D, Mun H, Ko S, Lee JW, Jathar S, Smith AS, Yao Y, Giang NT, Vu HH, Yan VC, Bridges MC, Kourtidis A, Muller F, Chang JH, Song SJ, Nakagawa S, Hirose T, Yoon JH, Song MS
Cell metabolism 2021 Dec 7;33(12):2380-2397.e9
Cell metabolism 2021 Dec 7;33(12):2380-2397.e9
Defective cyclophilin A induces TDP-43 proteinopathy: implications for amyotrophic lateral sclerosis and frontotemporal dementia.
Pasetto L, Grassano M, Pozzi S, Luotti S, Sammali E, Migazzi A, Basso M, Spagnolli G, Biasini E, Micotti E, Cerovic M, Carli M, Forloni G, De Marco G, Manera U, Moglia C, Mora G, Traynor BJ, Chiò A, Calvo A, Bonetto V
Brain : a journal of neurology 2021 Dec 31;144(12):3710-3726
Brain : a journal of neurology 2021 Dec 31;144(12):3710-3726
CellFIE: CRISPR- and Cell Fusion-based Two-hybrid Interaction Mapping of Endogenous Proteins.
Secker C, Kostova S, Niederlechner H, Beetz S, Wendland I, Liebich MJ, Polzer O, Groh M, Schnoegl S, Trepte P, Wanker EE
Journal of molecular biology 2021 Dec 3;433(24):167305
Journal of molecular biology 2021 Dec 3;433(24):167305
Circ-Hdgfrp3 shuttles along neurites and is trapped in aggregates formed by ALS-associated mutant FUS.
D'Ambra E, Santini T, Vitiello E, D'Uva S, Silenzi V, Morlando M, Bozzoni I
iScience 2021 Dec 17;24(12):103504
iScience 2021 Dec 17;24(12):103504
TDP-43 stabilizes G3BP1 mRNA: relevance to amyotrophic lateral sclerosis/frontotemporal dementia.
Sidibé H, Khalfallah Y, Xiao S, Gómez NB, Fakim H, Tank EMH, Di Tomasso G, Bareke E, Aulas A, McKeever PM, Melamed Z, Destroimaisons L, Deshaies JE, Zinman L, Parker JA, Legault P, Tétreault M, Barmada SJ, Robertson J, Vande Velde C
Brain : a journal of neurology 2021 Dec 16;144(11):3461-3476
Brain : a journal of neurology 2021 Dec 16;144(11):3461-3476
Novel chemical inhibitor against SOD1 misfolding and aggregation protects neuron-loss and ameliorates disease symptoms in ALS mouse model.
Woo TG, Yoon MH, Kang SM, Park S, Cho JH, Hwang YJ, Ahn J, Jang H, Shin YJ, Jung EM, Ha NC, Kim BH, Kwon Y, Park BJ
Communications biology 2021 Dec 15;4(1):1397
Communications biology 2021 Dec 15;4(1):1397
Drivers of Hsp104 potentiation revealed by scanning mutagenesis of the middle domain.
Ryan JJ, Bao A, Bell B, Ling C, Jackrel ME
Protein science : a publication of the Protein Society 2021 Aug;30(8):1667-1685
Protein science : a publication of the Protein Society 2021 Aug;30(8):1667-1685
Phosphorylation of trans-active response DNA-binding protein-of 43 kDa promotes its cytoplasmic aggregation and modulates its function in tau mRNA stability and exon 10 alternative splicing.
Wu R, Zhou D, Shen X, Chen F, Liu F, Gu J
Journal of neurochemistry 2021 Aug;158(3):766-778
Journal of neurochemistry 2021 Aug;158(3):766-778
Clinical and neuropathological diversity of tauopathy in MAPT duplication carriers.
Wallon D, Boluda S, Rovelet-Lecrux A, Thierry M, Lagarde J, Miguel L, Lecourtois M, Bonnevalle A, Sarazin M, Bottlaender M, Mula M, Marty S, Nakamura N, Schramm C, Sellal F, Jonveaux T, Heitz C, Le Ber I, Epelbaum S, Magnin E, Zarea A, Rousseau S, Quenez O, Hannequin D, Clavaguera F, Campion D, Duyckaerts C, Nicolas G
Acta neuropathologica 2021 Aug;142(2):259-278
Acta neuropathologica 2021 Aug;142(2):259-278
Tideglusib, a Non-ATP Competitive Inhibitor of GSK-3β as a Drug Candidate for the Treatment of Amyotrophic Lateral Sclerosis.
Martínez-González L, Gonzalo-Consuegra C, Gómez-Almería M, Porras G, de Lago E, Martín-Requero Á, Martínez A
International journal of molecular sciences 2021 Aug 20;22(16)
International journal of molecular sciences 2021 Aug 20;22(16)
Haploinsufficiency of the TDP43 ubiquitin E3 ligase RNF220 leads to ALS-like motor neuron defects in the mouse.
Ma P, Li Y, Wang H, Mao B
Journal of molecular cell biology 2021 Aug 18;13(5):374-382
Journal of molecular cell biology 2021 Aug 18;13(5):374-382
Extensive phenotypic characterisation of a human TDP-43(Q331K) transgenic mouse model of amyotrophic lateral sclerosis (ALS).
Watkins JA, Alix JJP, Shaw PJ, Mead RJ
Scientific reports 2021 Aug 17;11(1):16659
Scientific reports 2021 Aug 17;11(1):16659
A ligand-insensitive UNC5B splicing isoform regulates angiogenesis by promoting apoptosis.
Pradella D, Deflorian G, Pezzotta A, Di Matteo A, Belloni E, Campolungo D, Paradisi A, Bugatti M, Vermi W, Campioni M, Chiapparino A, Scietti L, Forneris F, Giampietro C, Volf N, Rehman M, Zacchigna S, Paronetto MP, Pistocchi A, Eichmann A, Mehlen P, Ghigna C
Nature communications 2021 Aug 11;12(1):4872
Nature communications 2021 Aug 11;12(1):4872
Decoding distinctive features of plasma extracellular vesicles in amyotrophic lateral sclerosis.
Pasetto L, Callegaro S, Corbelli A, Fiordaliso F, Ferrara D, Brunelli L, Sestito G, Pastorelli R, Bianchi E, Cretich M, Chiari M, Potrich C, Moglia C, Corbo M, Sorarù G, Lunetta C, Calvo A, Chiò A, Mora G, Pennuto M, Quattrone A, Rinaldi F, D'Agostino VG, Basso M, Bonetto V
Molecular neurodegeneration 2021 Aug 10;16(1):52
Molecular neurodegeneration 2021 Aug 10;16(1):52
Targeting TDP-43 Pathology Alleviates Cognitive and Motor Deficits Caused by Chronic Cerebral Hypoperfusion.
Thammisetty SS, Renaud L, Picher-Martel V, Weng YC, Calon F, Saikali S, Julien JP, Kriz J
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 2021 Apr;18(2):1095-1112
Neurotherapeutics : the journal of the American Society for Experimental NeuroTherapeutics 2021 Apr;18(2):1095-1112
Enhanced detection of expanded repeat mRNA foci with hybridization chain reaction.
Glineburg MR, Zhang Y, Krans A, Tank EM, Barmada SJ, Todd PK
Acta neuropathologica communications 2021 Apr 23;9(1):73
Acta neuropathologica communications 2021 Apr 23;9(1):73
Characterization of the upstream and intron promoters of the gene encoding TAR DNA-binding protein.
Hasegawa-Ogawa M, Okano HJ
Scientific reports 2021 Apr 22;11(1):8720
Scientific reports 2021 Apr 22;11(1):8720
TDP-43 prevents endogenous RNAs from triggering a lethal RIG-I-dependent interferon response.
Dunker W, Ye X, Zhao Y, Liu L, Richardson A, Karijolich J
Cell reports 2021 Apr 13;35(2):108976
Cell reports 2021 Apr 13;35(2):108976
Neuropathological and behavioral characterization of aged Grn R493X progranulin-deficient frontotemporal dementia knockin mice.
Frew J, Nygaard HB
Acta neuropathologica communications 2021 Apr 1;9(1):57
Acta neuropathologica communications 2021 Apr 1;9(1):57
The Role of HDAC6 in TDP-43-Induced Neurotoxicity and UPS Impairment.
Lee S, Kwon Y, Kim S, Jo M, Jeon YM, Cheon M, Lee S, Kim SR, Kim K, Kim HJ
Frontiers in cell and developmental biology 2020;8:581942
Frontiers in cell and developmental biology 2020;8:581942
Pathological Correlations of Neuropsychiatric Symptoms in Institutionalized People with Dementia.
Esteban de Antonio E, López-Álvarez J, Rábano A, Agüera-Ortiz L, Sánchez-Soblechero A, Amaya L, Portela S, Cátedra C, Olazarán J
Journal of Alzheimer's disease : JAD 2020;78(4):1731-1741
Journal of Alzheimer's disease : JAD 2020;78(4):1731-1741
The Contribution of Vascular Pathology Toward Cognitive Impairment in Older Individuals with Intermediate Braak Stage Tau Pathology.
Robinson AC, Roncaroli F, Chew-Graham S, Davidson YS, Minshull J, Horan MA, Payton A, Pendleton N, Mann DMA
Journal of Alzheimer's disease : JAD 2020;77(3):1005-1015
Journal of Alzheimer's disease : JAD 2020;77(3):1005-1015
TDP-43 Related Neuropathologies and Phosphorylation State: Associations with Age and Clinical Dementia in the Cambridge City over-75s Cohort.
Hunter S, Hokkanen SRK, Keage HAD, Fleming J, Minett T, Polvikoski T, Allinson K, Brayne C, Cambridge City over 75s Cohort collaboration
Journal of Alzheimer's disease : JAD 2020;75(1):337-350
Journal of Alzheimer's disease : JAD 2020;75(1):337-350
A Comparative Study of Pathological Outcomes in The University of Manchester Longitudinal Study of Cognition in Normal Healthy Old Age and Brains for Dementia Research Cohorts.
Robinson AC, Chew-Graham S, Davidson YS, Horan MA, Roncaroli F, Minshull J, du Plessis D, Pal P, Payton A, Pendleton N, Mann DMA
Journal of Alzheimer's disease : JAD 2020;73(2):619-632
Journal of Alzheimer's disease : JAD 2020;73(2):619-632
Functional analysis of proposed substrate-binding residues of Hsp104.
Howard MK, Sohn BS, von Borcke J, Xu A, Jackrel ME
PloS one 2020;15(3):e0230198
PloS one 2020;15(3):e0230198
Neurodegeneration-Associated Proteins in Human Olfactory Neurons Collected by Nasal Brushing.
Brozzetti L, Sacchetto L, Cecchini MP, Avesani A, Perra D, Bongianni M, Portioli C, Scupoli M, Ghetti B, Monaco S, Buffelli M, Zanusso G
Frontiers in neuroscience 2020;14:145
Frontiers in neuroscience 2020;14:145
Chaperone Mediated Autophagy Degrades TDP-43 Protein and Is Affected by TDP-43 Aggregation.
Ormeño F, Hormazabal J, Moreno J, Riquelme F, Rios J, Criollo A, Albornoz A, Alfaro IE, Budini M
Frontiers in molecular neuroscience 2020;13:19
Frontiers in molecular neuroscience 2020;13:19
Skeletal Muscle-Restricted Expression of Human SOD1 in Transgenic Mice Causes a Fatal ALS-Like Syndrome.
Martin LJ, Wong M
Frontiers in neurology 2020;11:592851
Frontiers in neurology 2020;11:592851
BV-2 Microglial Cells Overexpressing C9orf72 Hexanucleotide Repeat Expansion Produce DPR Proteins and Show Normal Functionality but No RNA Foci.
Rostalski H, Hietanen T, Leskelä S, Behánová A, Abdollahzadeh A, Wittrahm R, Mäkinen P, Huber N, Hoffmann D, Solje E, Remes AM, Natunen T, Takalo M, Tohka J, Hiltunen M, Haapasalo A
Frontiers in neurology 2020;11:550140
Frontiers in neurology 2020;11:550140
Modeling UBQLN2-mediated neurodegenerative disease in mice: Shared and divergent properties of wild type and mutant UBQLN2 in phase separation, subcellular localization, altered proteostasis pathways, and selective cytotoxicity.
Sharkey LM, Sandoval-Pistorius SS, Moore SJ, Gerson JE, Komlo R, Fischer S, Negron-Rios KY, Crowley EV, Padron F, Patel R, Murphy GG, Paulson HL
Neurobiology of disease 2020 Sep;143:105016
Neurobiology of disease 2020 Sep;143:105016
Impaired NHEJ repair in amyotrophic lateral sclerosis is associated with TDP-43 mutations.
Konopka A, Whelan DR, Jamali MS, Perri E, Shahheydari H, Toth RP, Parakh S, Robinson T, Cheong A, Mehta P, Vidal M, Ragagnin AMG, Khizhnyak I, Jagaraj CJ, Galper J, Grima N, Deva A, Shadfar S, Nicholson GA, Yang S, Cutts SM, Horejsi Z, Bell TDM, Walker AK, Blair IP, Atkin JD
Molecular neurodegeneration 2020 Sep 9;15(1):51
Molecular neurodegeneration 2020 Sep 9;15(1):51
Isolation and characterization of antibody fragments selective for human FTD brain derived TDP-43 variants.
Venkataraman L, He P, Khan G, Harris BT, Sierks MR
BMC neuroscience 2020 Sep 4;21(1):36
BMC neuroscience 2020 Sep 4;21(1):36
Conditioned Medium from Cells Overexpressing TDP-43 Alters the Metabolome of Recipient Cells.
Hergesheimer R, Lanznaster D, Bourgeais J, Hérault O, Vourc'h P, Andres CR, Corcia P, Blasco H
Cells 2020 Sep 29;9(10)
Cells 2020 Sep 29;9(10)
TDP-43 mediated blood-brain barrier permeability and leukocyte infiltration promote neurodegeneration in a low-grade systemic inflammation mouse model.
Zamudio F, Loon AR, Smeltzer S, Benyamine K, Navalpur Shanmugam NK, Stewart NJF, Lee DC, Nash K, Selenica MB
Journal of neuroinflammation 2020 Sep 26;17(1):283
Journal of neuroinflammation 2020 Sep 26;17(1):283
Stress Granule Assembly Can Facilitate but Is Not Required for TDP-43 Cytoplasmic Aggregation.
Fernandes N, Nero L, Lyons SM, Ivanov P, Mittelmeier TM, Bolger TA, Buchan JR
Biomolecules 2020 Sep 25;10(10)
Biomolecules 2020 Sep 25;10(10)
G(4)C(2) Repeat RNA Initiates a POM121-Mediated Reduction in Specific Nucleoporins in C9orf72 ALS/FTD.
Coyne AN, Zaepfel BL, Hayes L, Fitchman B, Salzberg Y, Luo EC, Bowen K, Trost H, Aigner S, Rigo F, Yeo GW, Harel A, Svendsen CN, Sareen D, Rothstein JD
Neuron 2020 Sep 23;107(6):1124-1140.e11
Neuron 2020 Sep 23;107(6):1124-1140.e11
The effect and mechanism of 19S proteasome PSMD11/Rpn6 subunit in D-Galactose induced mimetic aging models.
Wu H, Sun H, He Z, Chen X, Li Y, Zhao X, Kong W, Kong W
Experimental cell research 2020 Sep 1;394(1):112093
Experimental cell research 2020 Sep 1;394(1):112093
Absence of Subcerebral Projection Neurons Is Beneficial in a Mouse Model of Amyotrophic Lateral Sclerosis.
Burg T, Bichara C, Scekic-Zahirovic J, Fischer M, Stuart-Lopez G, Brunet A, Lefebvre F, Cordero-Erausquin M, Rouaux C
Annals of neurology 2020 Oct;88(4):688-702
Annals of neurology 2020 Oct;88(4):688-702
Cytoplasmic localization of amyotrophic lateral sclerosis-related TDP-43 proteins modulates stress granule formation.
Besnard-Guérin C
The European journal of neuroscience 2020 Oct;52(8):3995-4008
The European journal of neuroscience 2020 Oct;52(8):3995-4008
Nucleated transcriptional condensates amplify gene expression.
Wei MT, Chang YC, Shimobayashi SF, Shin Y, Strom AR, Brangwynne CP
Nature cell biology 2020 Oct;22(10):1187-1196
Nature cell biology 2020 Oct;22(10):1187-1196
An ALS-linked mutation in TDP-43 disrupts normal protein interactions in the motor neuron response to oxidative stress.
Feneberg E, Gordon D, Thompson AG, Finelli MJ, Dafinca R, Candalija A, Charles PD, Mäger I, Wood MJ, Fischer R, Kessler BM, Gray E, Turner MR, Talbot K
Neurobiology of disease 2020 Oct;144:105050
Neurobiology of disease 2020 Oct;144:105050
The overexpression of TDP-43 in astrocytes causes neurodegeneration via a PTP1B-mediated inflammatory response.
Lee S, Kim S, Kang HY, Lim HR, Kwon Y, Jo M, Jeon YM, Kim SR, Kim K, Ha CM, Lee S, Kim HJ
Journal of neuroinflammation 2020 Oct 14;17(1):299
Journal of neuroinflammation 2020 Oct 14;17(1):299
Spinal Cord and Motor Neuron TDP-43 Pathology in a Sporadic Inclusion Body Myositis Patient.
Cathcart SJ, Greene EP, Powell SZ, Arumanayagam AS, Rivera AL, Tawil R, Appel SH, Cykowski MD
Journal of neuropathology and experimental neurology 2020 Oct 1;79(10):1130-1133
Journal of neuropathology and experimental neurology 2020 Oct 1;79(10):1130-1133
Sensitivity-Specificity of Tau and Amyloid β Positron Emission Tomography in Frontotemporal Lobar Degeneration.
Ghirelli A, Tosakulwong N, Weigand SD, Clark HM, Ali F, Botha H, Duffy JR, Utianski RL, Buciuc M, Murray ME, Labuzan SA, Spychalla AJ, Pham NTT, Schwarz CG, Senjem ML, Machulda MM, Baker M, Rademakers R, Filippi M, Jack CR Jr, Lowe VJ, Parisi JE, Dickson DW, Josephs KA, Whitwell JL
Annals of neurology 2020 Nov;88(5):1009-1022
Annals of neurology 2020 Nov;88(5):1009-1022
Multiplexed chemogenetics in astrocytes and motoneurons restore blood-spinal cord barrier in ALS.
Ouali Alami N, Tang L, Wiesner D, Commisso B, Bayer D, Weishaupt J, Dupuis L, Wong P, Baumann B, Wirth T, Boeckers TM, Yilmazer-Hanke D, Ludolph A, Roselli F
Life science alliance 2020 Nov;3(11)
Life science alliance 2020 Nov;3(11)
Cytoplasmic mislocalization and mitochondrial colocalization of TDP-43 are common features between normal aged and young mice.
Termsarasab P, Thammongkolchai T, Gao J, Wang L, Liang J, Wang X
Experimental biology and medicine (Maywood, N.J.) 2020 Nov;245(17):1584-1593
Experimental biology and medicine (Maywood, N.J.) 2020 Nov;245(17):1584-1593
TDP-43 pathology in primary lateral sclerosis.
Mackenzie IRA, Briemberg H
Amyotrophic lateral sclerosis & frontotemporal degeneration 2020 Nov;21(sup1):52-58
Amyotrophic lateral sclerosis & frontotemporal degeneration 2020 Nov;21(sup1):52-58
Cognitive deficit, and neuropathological correlates, in the oldest-old.
Verny M, Duyckaerts C
Revue neurologique 2020 Nov;176(9):670-676
Revue neurologique 2020 Nov;176(9):670-676
Chronic stress induces formation of stress granules and pathological TDP-43 aggregates in human ALS fibroblasts and iPSC-motoneurons.
Ratti A, Gumina V, Lenzi P, Bossolasco P, Fulceri F, Volpe C, Bardelli D, Pregnolato F, Maraschi A, Fornai F, Silani V, Colombrita C
Neurobiology of disease 2020 Nov;145:105051
Neurobiology of disease 2020 Nov;145:105051
TDP-43 transports ribosomal protein mRNA to regulate axonal local translation in neuronal axons.
Nagano S, Jinno J, Abdelhamid RF, Jin Y, Shibata M, Watanabe S, Hirokawa S, Nishizawa M, Sakimura K, Onodera O, Okada H, Okada T, Saito Y, Takahashi-Fujigasaki J, Murayama S, Wakatsuki S, Mochizuki H, Araki T
Acta neuropathologica 2020 Nov;140(5):695-713
Acta neuropathologica 2020 Nov;140(5):695-713
Antibody against TDP-43 phosphorylated at serine 375 suggests conformational differences of TDP-43 aggregates among FTLD-TDP subtypes.
Neumann M, Frick P, Paron F, Kosten J, Buratti E, Mackenzie IR
Acta neuropathologica 2020 Nov;140(5):645-658
Acta neuropathologica 2020 Nov;140(5):645-658
TDP-43 interacts with amyloid-β, inhibits fibrillization, and worsens pathology in a model of Alzheimer's disease.
Shih YH, Tu LH, Chang TY, Ganesan K, Chang WW, Chang PS, Fang YS, Lin YT, Jin LW, Chen YR
Nature communications 2020 Nov 23;11(1):5950
Nature communications 2020 Nov 23;11(1):5950
Loss of TDP-43 in astrocytes leads to motor deficits by triggering A1-like reactive phenotype and triglial dysfunction.
Peng AYT, Agrawal I, Ho WY, Yen YC, Pinter AJ, Liu J, Phua QXC, Koh KB, Chang JC, Sanford E, Man JHK, Wong P, Gutmann DH, Tucker-Kellogg G, Ling SC
Proceedings of the National Academy of Sciences of the United States of America 2020 Nov 17;117(46):29101-29112
Proceedings of the National Academy of Sciences of the United States of America 2020 Nov 17;117(46):29101-29112
TDP-43 proteinopathies: a new wave of neurodegenerative diseases.
de Boer EMJ, Orie VK, Williams T, Baker MR, De Oliveira HM, Polvikoski T, Silsby M, Menon P, van den Bos M, Halliday GM, van den Berg LH, Van Den Bosch L, van Damme P, Kiernan MC, van Es MA, Vucic S
Journal of neurology, neurosurgery, and psychiatry 2020 Nov 11;92(1):86-95
Journal of neurology, neurosurgery, and psychiatry 2020 Nov 11;92(1):86-95
Novel monoclonal antibodies targeting the RRM2 domain of human TDP-43 protein.
Trejo-Lopez JA, Sorrentino ZA, Riffe CJ, Lloyd GM, Labuzan SA, Dickson DW, Yachnis AT, Prokop S, Giasson BI
Neuroscience letters 2020 Nov 1;738:135353
Neuroscience letters 2020 Nov 1;738:135353
RNA-binding protein altered expression and mislocalization in MS.
Masaki K, Sonobe Y, Ghadge G, Pytel P, Lépine P, Pernin F, Cui QL, Antel JP, Zandee S, Prat A, Roos RP
Neurology(R) neuroimmunology & neuroinflammation 2020 May;7(3)
Neurology(R) neuroimmunology & neuroinflammation 2020 May;7(3)
Mutant C9orf72 human iPSC-derived astrocytes cause non-cell autonomous motor neuron pathophysiology.
Zhao C, Devlin AC, Chouhan AK, Selvaraj BT, Stavrou M, Burr K, Brivio V, He X, Mehta AR, Story D, Shaw CE, Dando O, Hardingham GE, Miles GB, Chandran S
Glia 2020 May;68(5):1046-1064
Glia 2020 May;68(5):1046-1064
ALS-derived fibroblasts exhibit reduced proliferation rate, cytoplasmic TDP-43 aggregation and a higher susceptibility to DNA damage.
Riancho J, Castanedo-Vázquez D, Gil-Bea F, Tapia O, Arozamena J, Durán-Vían C, Sedano MJ, Berciano MT, Lopez de Munain A, Lafarga M
Journal of neurology 2020 May;267(5):1291-1299
Journal of neurology 2020 May;267(5):1291-1299
Transmission of ALS pathogenesis by the cerebrospinal fluid.
Mishra PS, Boutej H, Soucy G, Bareil C, Kumar S, Picher-Martel V, Dupré N, Kriz J, Julien JP
Acta neuropathologica communications 2020 May 7;8(1):65
Acta neuropathologica communications 2020 May 7;8(1):65
HDAC1 inhibition ameliorates TDP-43-induced cell death in vitro and in vivo.
Sanna S, Esposito S, Masala A, Sini P, Nieddu G, Galioto M, Fais M, Iaccarino C, Cestra G, Crosio C
Cell death & disease 2020 May 14;11(5):369
Cell death & disease 2020 May 14;11(5):369
A role for SUMOylation in the Formation and Cellular Localization of TDP-43 Aggregates in Amyotrophic Lateral Sclerosis.
Maurel C, Chami AA, Thépault RA, Marouillat S, Blasco H, Corcia P, Andres CR, Vourc'h P
Molecular neurobiology 2020 Mar;57(3):1361-1373
Molecular neurobiology 2020 Mar;57(3):1361-1373
Elevation of casein kinase 1ε associated with TDP-43 and tau pathologies in Alzheimer's disease.
Gu J, Hu W, Tan X, Qu S, Chu D, Gong CX, Iqbal K, Liu F
Brain pathology (Zurich, Switzerland) 2020 Mar;30(2):283-297
Brain pathology (Zurich, Switzerland) 2020 Mar;30(2):283-297
Sequestosome-1 (p62) expression reveals chaperone-assisted selective autophagy in immune-mediated necrotizing myopathies.
Fischer N, Preuße C, Radke J, Pehl D, Allenbach Y, Schneider U, Feist E, von Casteleyn V, Hahn K, Ruck T, Meuth SG, Goebel HH, Graf R, Mammen A, Benveniste O, Stenzel W
Brain pathology (Zurich, Switzerland) 2020 Mar;30(2):261-271
Brain pathology (Zurich, Switzerland) 2020 Mar;30(2):261-271
Forced isoform switching of Neat1_1 to Neat1_2 leads to the loss of Neat1_1 and the hyperformation of paraspeckles but does not affect the development and growth of mice.
Isobe M, Toya H, Mito M, Chiba T, Asahara H, Hirose T, Nakagawa S
RNA (New York, N.Y.) 2020 Mar;26(3):251-264
RNA (New York, N.Y.) 2020 Mar;26(3):251-264
Human subiculo-fornico-mamillary system in Alzheimer's disease: Tau seeding by the pillar of the fornix.
Thierry M, Boluda S, Delatour B, Marty S, Seilhean D, Brainbank Neuro-CEB Neuropathology Network, Potier MC, Duyckaerts C
Acta neuropathologica 2020 Mar;139(3):443-461
Acta neuropathologica 2020 Mar;139(3):443-461
The SINEB1 element in the long non-coding RNA Malat1 is necessary for TDP-43 proteostasis.
Nguyen TM, Kabotyanski EB, Reineke LC, Shao J, Xiong F, Lee JH, Dubrulle J, Johnson H, Stossi F, Tsoi PS, Choi KJ, Ellis AG, Zhao N, Cao J, Adewunmi O, Ferreon JC, Ferreon ACM, Neilson JR, Mancini MA, Chen X, Kim J, Ma L, Li W, Rosen JM
Nucleic acids research 2020 Mar 18;48(5):2621-2642
Nucleic acids research 2020 Mar 18;48(5):2621-2642
Sigma-1 receptor is a key genetic modulator in amyotrophic lateral sclerosis.
Couly S, Khalil B, Viguier V, Roussel J, Maurice T, Liévens JC
Human molecular genetics 2020 Mar 13;29(4):529-540
Human molecular genetics 2020 Mar 13;29(4):529-540
Motor neuron preservation and decrease of in vivo TDP-43 phosphorylation by protein CK-1δ kinase inhibitor treatment.
Martínez-González L, Rodríguez-Cueto C, Cabezudo D, Bartolomé F, Andrés-Benito P, Ferrer I, Gil C, Martín-Requero Á, Fernández-Ruiz J, Martínez A, de Lago E
Scientific reports 2020 Mar 10;10(1):4449
Scientific reports 2020 Mar 10;10(1):4449
CYLD is a causative gene for frontotemporal dementia - amyotrophic lateral sclerosis.
Dobson-Stone C, Hallupp M, Shahheydari H, Ragagnin AMG, Chatterton Z, Carew-Jones F, Shepherd CE, Stefen H, Paric E, Fath T, Thompson EM, Blumbergs P, Short CL, Field CD, Panegyres PK, Hecker J, Nicholson G, Shaw AD, Fullerton JM, Luty AA, Schofield PR, Brooks WS, Rajan N, Bennett MF, Bahlo M, Landers JE, Piguet O, Hodges JR, Halliday GM, Topp SD, Smith BN, Shaw CE, McCann E, Fifita JA, Williams KL, Atkin JD, Blair IP, Kwok JB
Brain : a journal of neurology 2020 Mar 1;143(3):783-799
Brain : a journal of neurology 2020 Mar 1;143(3):783-799
Clinical and pathologic phenotype of a large family with heterozygous STUB1 mutation.
Mol MO, van Rooij JGJ, Brusse E, Verkerk AJMH, Melhem S, den Dunnen WFA, Rizzu P, Cupidi C, van Swieten JC, Donker Kaat L
Neurology. Genetics 2020 Jun;6(3):e417
Neurology. Genetics 2020 Jun;6(3):e417
Mutant TDP-43 Expression Triggers TDP-43 Pathology and Cell Autonomous Effects on Primary Astrocytes: Implications for Non-cell Autonomous Pathology in ALS.
Barton SK, Lau CL, Chiam MDF, Tomas D, Muyderman H, Beart PM, Turner BJ
Neurochemical research 2020 Jun;45(6):1451-1459
Neurochemical research 2020 Jun;45(6):1451-1459
Endoplasmic Reticulum Stress Signalling Induces Casein Kinase 1-Dependent Formation of Cytosolic TDP-43 Inclusions in Motor Neuron-Like Cells.
Hicks DA, Cross LL, Williamson R, Rattray M
Neurochemical research 2020 Jun;45(6):1354-1364
Neurochemical research 2020 Jun;45(6):1354-1364
Diagnostic and prognostic values of PBMC proteins in amyotrophic lateral sclerosis.
Luotti S, Pasetto L, Porcu L, Torri V, Elezgarai SR, Pantalone S, Filareti M, Corbo M, Lunetta C, Mora G, Bonetto V
Neurobiology of disease 2020 Jun;139:104815
Neurobiology of disease 2020 Jun;139:104815
HDAC1 dysregulation induces aberrant cell cycle and DNA damage in progress of TDP-43 proteinopathies.
Wu CC, Jin LW, Wang IF, Wei WY, Ho PC, Liu YC, Tsai KJ
EMBO molecular medicine 2020 Jun 8;12(6):e10622
EMBO molecular medicine 2020 Jun 8;12(6):e10622
RBM45 associates with nuclear stress bodies and forms nuclear inclusions during chronic cellular stress and in neurodegenerative diseases.
Collins M, Li Y, Bowser R
Acta neuropathologica communications 2020 Jun 26;8(1):91
Acta neuropathologica communications 2020 Jun 26;8(1):91
Mitigation of ALS Pathology by Neuron-Specific Inhibition of Nuclear Factor Kappa B Signaling.
Dutta K, Thammisetty SS, Boutej H, Bareil C, Julien JP
The Journal of neuroscience : the official journal of the Society for Neuroscience 2020 Jun 24;40(26):5137-5154
The Journal of neuroscience : the official journal of the Society for Neuroscience 2020 Jun 24;40(26):5137-5154
TDP-43, a protein central to amyotrophic lateral sclerosis, is destabilized by tankyrase-1 and -2.
McGurk L, Rifai OM, Bonini NM
Journal of cell science 2020 Jun 23;133(12)
Journal of cell science 2020 Jun 23;133(12)
Extracellular Vesicles Isolated from Human Induced Pluripotent Stem Cell-Derived Neurons Contain a Transcriptional Network.
Hicks DA, Jones AC, Corbett NJ, Fisher K, Pickering-Brown SM, Ashe MP, Hooper NM
Neurochemical research 2020 Jul;45(7):1711-1728
Neurochemical research 2020 Jul;45(7):1711-1728
SOD1 p.D12Y variant is associated with amyotrophic lateral sclerosis/distal myopathy spectrum.
Tasca G, Lattante S, Marangi G, Conte A, Bernardo D, Bisogni G, Mandich P, Zollino M, Ragozzino E, Udd B, Sabatelli M
European journal of neurology 2020 Jul;27(7):1304-1309
European journal of neurology 2020 Jul;27(7):1304-1309
Amyotrophic Lateral Sclerosis Modifiers in Drosophila Reveal the Phospholipase D Pathway as a Potential Therapeutic Target.
Kankel MW, Sen A, Lu L, Theodorou M, Dimlich DN, McCampbell A, Henderson CE, Shneider NA, Artavanis-Tsakonas S
Genetics 2020 Jul;215(3):747-766
Genetics 2020 Jul;215(3):747-766
The Impact of ALS-Associated Genes hnRNPA1, MATR3, VCP and UBQLN2 on the Severity of TDP-43 Aggregation.
Bajc Česnik A, Motaln H, Rogelj B
Cells 2020 Jul 28;9(8)
Cells 2020 Jul 28;9(8)
DeepCLIP: predicting the effect of mutations on protein-RNA binding with deep learning.
Grønning AGB, Doktor TK, Larsen SJ, Petersen USS, Holm LL, Bruun GH, Hansen MB, Hartung AM, Baumbach J, Andresen BS
Nucleic acids research 2020 Jul 27;48(13):7099-7118
Nucleic acids research 2020 Jul 27;48(13):7099-7118
Modeling of Frontotemporal Dementia Using iPSC Technology.
Kim M, Kim HJ, Koh W, Li L, Heo H, Cho H, Lyoo CH, Seo SW, Kim EJ, Nakanishi M, Na DL, Song J
International journal of molecular sciences 2020 Jul 27;21(15)
International journal of molecular sciences 2020 Jul 27;21(15)
Loss of Tdp-43 disrupts the axonal transcriptome of motoneurons accompanied by impaired axonal translation and mitochondria function.
Briese M, Saal-Bauernschubert L, Lüningschrör P, Moradi M, Dombert B, Surrey V, Appenzeller S, Deng C, Jablonka S, Sendtner M
Acta neuropathologica communications 2020 Jul 24;8(1):116
Acta neuropathologica communications 2020 Jul 24;8(1):116
Chimeric Peptide Species Contribute to Divergent Dipeptide Repeat Pathology in c9ALS/FTD and SCA36.
McEachin ZT, Gendron TF, Raj N, García-Murias M, Banerjee A, Purcell RH, Ward PJ, Todd TW, Merritt-Garza ME, Jansen-West K, Hales CM, García-Sobrino T, Quintáns B, Holler CJ, Taylor G, San Millán B, Teijeira S, Yamashita T, Ohkubo R, Boulis NM, Xu C, Wen Z, Streichenberger N, Neuro–CEB Neuropathology Network, Fogel BL, Kukar T, Abe K, Dickson DW, Arias M, Glass JD, Jiang J, Tansey MG, Sobrido MJ, Petrucelli L, Rossoll W, Bassell GJ
Neuron 2020 Jul 22;107(2):292-305.e6
Neuron 2020 Jul 22;107(2):292-305.e6
Revealing the Proteome of Motor Cortex Derived Extracellular Vesicles Isolated from Amyotrophic Lateral Sclerosis Human Postmortem Tissues.
Vassileff N, Vella LJ, Rajapaksha H, Shambrook M, Kenari AN, McLean C, Hill AF, Cheng L
Cells 2020 Jul 16;9(7)
Cells 2020 Jul 16;9(7)
Glutathione S-Transferase Rescues Motor Neuronal Toxicity in Fly Model of Amyotrophic Lateral Sclerosis.
Cha SJ, Han YJ, Choi HJ, Kim HJ, Kim K
Antioxidants (Basel, Switzerland) 2020 Jul 14;9(7)
Antioxidants (Basel, Switzerland) 2020 Jul 14;9(7)
Expanding the Phenotype of Frontotemporal Lobar Degeneration With FUS-Positive Pathology (FTLD-FUS).
Chornenka K, Hirsch-Reinshagen V, Perez-Rosendahl M, Feldman H, Segal-Gidan F, Vinters HV, Mackenzie IR
Journal of neuropathology and experimental neurology 2020 Jul 1;79(7):809-812
Journal of neuropathology and experimental neurology 2020 Jul 1;79(7):809-812
Subcortical TDP-43 pathology patterns validate cortical FTLD-TDP subtypes and demonstrate unique aspects of C9orf72 mutation cases.
Mackenzie IR, Neumann M
Acta neuropathologica 2020 Jan;139(1):83-98
Acta neuropathologica 2020 Jan;139(1):83-98
Overexpression of human wtTDP-43 causes impairment in hippocampal plasticity and behavioral deficits in CAMKII-tTa transgenic mouse model.
Quadri Z, Johnson N, Zamudio F, Miller A, Peters M, Smeltzer S, Hunt JB Jr, Housley SB, Brown B, Kraner S, Norris CM, Nash K, Weeber E, Lee DC, Selenica MB
Molecular and cellular neurosciences 2020 Jan;102:103418
Molecular and cellular neurosciences 2020 Jan;102:103418
Cdc48/VCP and Endocytosis Regulate TDP-43 and FUS Toxicity and Turnover.
Liu G, Byrd A, Warner AN, Pei F, Basha E, Buchanan A, Buchan JR
Molecular and cellular biology 2020 Jan 30;40(4)
Molecular and cellular biology 2020 Jan 30;40(4)
Changes to the TDP-43 and FUS Interactomes Induced by DNA Damage.
Kawaguchi T, Rollins MG, Moinpour M, Morera AA, Ebmeier CC, Old WM, Schwartz JC
Journal of proteome research 2020 Jan 3;19(1):360-370
Journal of proteome research 2020 Jan 3;19(1):360-370
A robust TDP-43 knock-in mouse model of ALS.
Huang SL, Wu LS, Lee M, Chang CW, Cheng WC, Fang YS, Chen YR, Cheng PL, Shen CJ
Acta neuropathologica communications 2020 Jan 21;8(1):3
Acta neuropathologica communications 2020 Jan 21;8(1):3
Multiple distinct pathways lead to hyperubiquitylated insoluble TDP-43 protein independent of its translocation into stress granules.
Hans F, Glasebach H, Kahle PJ
The Journal of biological chemistry 2020 Jan 17;295(3):673-689
The Journal of biological chemistry 2020 Jan 17;295(3):673-689
TDP-43 facilitates milk lipid secretion by post-transcriptional regulation of Btn1a1 and Xdh.
Zhao L, Ke H, Xu H, Wang GD, Zhang H, Zou L, Xiang S, Li M, Peng L, Zhou M, Li L, Ao L, Yang Q, Shen CJ, Yi P, Wang L, Jiao B
Nature communications 2020 Jan 17;11(1):341
Nature communications 2020 Jan 17;11(1):341
Structural and mechanistic insights into Hsp104 function revealed by synchrotron X-ray footprinting.
Sweeny EA, Tariq A, Gurpinar E, Go MS, Sochor MA, Kan ZY, Mayne L, Englander SW, Shorter J
The Journal of biological chemistry 2020 Feb 7;295(6):1517-1538
The Journal of biological chemistry 2020 Feb 7;295(6):1517-1538
Active poly-GA vaccination prevents microglia activation and motor deficits in a C9orf72 mouse model.
Zhou Q, Mareljic N, Michaelsen M, Parhizkar S, Heindl S, Nuscher B, Farny D, Czuppa M, Schludi C, Graf A, Krebs S, Blum H, Feederle R, Roth S, Haass C, Arzberger T, Liesz A, Edbauer D
EMBO molecular medicine 2020 Feb 7;12(2):e10919
EMBO molecular medicine 2020 Feb 7;12(2):e10919
Use of Capillary Electrophoresis Immunoassay to Search for Potential Biomarkers of Amyotrophic Lateral Sclerosis in Human Platelets.
Sage JM, Hall L, McVey A, Barohn RJ, Statland JM, Jawdat O, Dimachkie MM, Agbas A
Journal of visualized experiments : JoVE 2020 Feb 10;(156)
Journal of visualized experiments : JoVE 2020 Feb 10;(156)
From the Entorhinal Region via the Prosubiculum to the Dentate Fascia: Alzheimer Disease-Related Neurofibrillary Changes in the Temporal Allocortex.
Braak H, Del Tredici K
Journal of neuropathology and experimental neurology 2020 Feb 1;79(2):163-175
Journal of neuropathology and experimental neurology 2020 Feb 1;79(2):163-175
Neurotoxic microglia promote TDP-43 proteinopathy in progranulin deficiency.
Zhang J, Velmeshev D, Hashimoto K, Huang YH, Hofmann JW, Shi X, Chen J, Leidal AM, Dishart JG, Cahill MK, Kelley KW, Liddelow SA, Seeley WW, Miller BL, Walther TC, Farese RV Jr, Taylor JP, Ullian EM, Huang B, Debnath J, Wittmann T, Kriegstein AR, Huang EJ
Nature 2020 Dec;588(7838):459-465
Nature 2020 Dec;588(7838):459-465
An Experimental Model of Neurodegenerative Disease Based on Porcine Hemagglutinating Encephalomyelitis Virus-Related Lysosomal Abnormalities.
Lan Y, Li Z, Wang Z, Wang X, Wang G, Zhang J, Hu S, Zhao K, Xu B, Gao F, He W
Molecular neurobiology 2020 Dec;57(12):5299-5306
Molecular neurobiology 2020 Dec;57(12):5299-5306
Accumulation of phosphorylated TDP-43 in the cytoplasm of Schwann cells in a case of sporadic amyotrophic lateral sclerosis.
Nakamura-Shindo K, Sakai K, Shimizu A, Ishida C, Yamada M
Neuropathology : official journal of the Japanese Society of Neuropathology 2020 Dec;40(6):606-610
Neuropathology : official journal of the Japanese Society of Neuropathology 2020 Dec;40(6):606-610
LSM12-EPAC1 defines a neuroprotective pathway that sustains the nucleocytoplasmic RAN gradient.
Lee J, Park J, Kim JH, Lee G, Park TE, Yoon KJ, Kim YK, Lim C
PLoS biology 2020 Dec;18(12):e3001002
PLoS biology 2020 Dec;18(12):e3001002
TDP-43 mutations link Amyotrophic Lateral Sclerosis with R-loop homeostasis and R loop-mediated DNA damage.
Giannini M, Bayona-Feliu A, Sproviero D, Barroso SI, Cereda C, Aguilera A
PLoS genetics 2020 Dec;16(12):e1009260
PLoS genetics 2020 Dec;16(12):e1009260
Optogenetic TDP-43 nucleation induces persistent insoluble species and progressive motor dysfunction in vivo.
Otte CG, Fortuna TR, Mann JR, Gleixner AM, Ramesh N, Pyles NJ, Pandey UB, Donnelly CJ
Neurobiology of disease 2020 Dec;146:105078
Neurobiology of disease 2020 Dec;146:105078
Influence of APOE genotype in primary age-related tauopathy.
Robinson AC, Davidson YS, Roncaroli F, Minshull J, Tinkler P, Horan MA, Payton A, Pendleton N, Mann DMA
Acta neuropathologica communications 2020 Dec 7;8(1):215
Acta neuropathologica communications 2020 Dec 7;8(1):215
Analysis of ALS-related proteins during herpes simplex virus-2 latent infection.
Cabrera JR, Rodríguez-Izquierdo I, Jiménez JL, Muñoz-Fernández MÁ
Journal of neuroinflammation 2020 Dec 7;17(1):371
Journal of neuroinflammation 2020 Dec 7;17(1):371
18F-flortaucipir PET to autopsy comparisons in Alzheimer's disease and other neurodegenerative diseases.
Soleimani-Meigooni DN, Iaccarino L, La Joie R, Baker S, Bourakova V, Boxer AL, Edwards L, Eser R, Gorno-Tempini ML, Jagust WJ, Janabi M, Kramer JH, Lesman-Segev OH, Mellinger T, Miller BL, Pham J, Rosen HJ, Spina S, Seeley WW, Strom A, Grinberg LT, Rabinovici GD
Brain : a journal of neurology 2020 Dec 5;143(11):3477-3494
Brain : a journal of neurology 2020 Dec 5;143(11):3477-3494
Evidence of A Negative Feedback Network Between TDP-43 and miRNAs Dependent on TDP-43 Nuclear Localization.
Hawley ZCE, Campos-Melo D, Strong MJ
Journal of molecular biology 2020 Dec 4;432(24):166695
Journal of molecular biology 2020 Dec 4;432(24):166695
Nuclear Import Receptors Directly Bind to Arginine-Rich Dipeptide Repeat Proteins and Suppress Their Pathological Interactions.
Hutten S, Usluer S, Bourgeois B, Simonetti F, Odeh HM, Fare CM, Czuppa M, Hruska-Plochan M, Hofweber M, Polymenidou M, Shorter J, Edbauer D, Madl T, Dormann D
Cell reports 2020 Dec 22;33(12):108538
Cell reports 2020 Dec 22;33(12):108538
Calpain-1 and Calpain-2 in the Brain: New Evidence for a Critical Role of Calpain-2 in Neuronal Death.
Wang Y, Liu Y, Bi X, Baudry M
Cells 2020 Dec 16;9(12)
Cells 2020 Dec 16;9(12)
Therapeutic genetic variation revealed in diverse Hsp104 homologs.
March ZM, Sweeney K, Kim H, Yan X, Castellano LM, Jackrel ME, Lin J, Chuang E, Gomes E, Willicott CW, Michalska K, Jedrzejczak RP, Joachimiak A, Caldwell KA, Caldwell GA, Shalem O, Shorter J
eLife 2020 Dec 15;9
eLife 2020 Dec 15;9
Cytosolic calcium regulates cytoplasmic accumulation of TDP-43 through Calpain-A and Importin α3.
Park JH, Chung CG, Park SS, Lee D, Kim KM, Jeong Y, Kim ES, Cho JH, Jeon YM, Shen CJ, Kim HJ, Hwang D, Lee SB
eLife 2020 Dec 11;9
eLife 2020 Dec 11;9
The vulnerability of motor and frontal cortex-dependent behaviors in mice expressing ALS-linked mutation in TDP-43.
Wong P, Ho WY, Yen YC, Sanford E, Ling SC
Neurobiology of aging 2020 Aug;92:43-60
Neurobiology of aging 2020 Aug;92:43-60
A New Glycogen Storage Disease Caused by a Dominant PYGM Mutation.
Echaniz-Laguna A, Lornage X, Laforêt P, Orngreen MC, Edelweiss E, Brochier G, Bui MT, Silva-Rojas R, Birck C, Lannes B, Romero NB, Vissing J, Laporte J, Böhm J
Annals of neurology 2020 Aug;88(2):274-282
Annals of neurology 2020 Aug;88(2):274-282
All-Trans Retinoic Acid Exerts Neuroprotective Effects in Amyotrophic Lateral Sclerosis-Like Tg (SOD1*G93A)1Gur Mice.
Zhu Y, Liu Y, Yang F, Chen W, Jiang J, He P, Jiang S, Li M, Xu R
Molecular neurobiology 2020 Aug;57(8):3603-3615
Molecular neurobiology 2020 Aug;57(8):3603-3615
PTK2/FAK regulates UPS impairment via SQSTM1/p62 phosphorylation in TARDBP/TDP-43 proteinopathies.
Lee S, Jeon YM, Cha SJ, Kim S, Kwon Y, Jo M, Jang YN, Lee S, Kim J, Kim SR, Lee KJ, Lee SB, Kim K, Kim HJ
Autophagy 2020 Aug;16(8):1396-1412
Autophagy 2020 Aug;16(8):1396-1412
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
LaClair KD, Zhou Q, Michaelsen M, Wefers B, Brill MS, Janjic A, Rathkolb B, Farny D, Cygan M, de Angelis MH, Wurst W, Neumann M, Enard W, Misgeld T, Arzberger T, Edbauer D
Acta neuropathologica 2020 Aug;140(2):121-142
Acta neuropathologica 2020 Aug;140(2):121-142
Stress Induces Dynamic, Cytotoxicity-Antagonizing TDP-43 Nuclear Bodies via Paraspeckle LncRNA NEAT1-Mediated Liquid-Liquid Phase Separation.
Wang C, Duan Y, Duan G, Wang Q, Zhang K, Deng X, Qian B, Gu J, Ma Z, Zhang S, Guo L, Liu C, Fang Y
Molecular cell 2020 Aug 6;79(3):443-458.e7
Molecular cell 2020 Aug 6;79(3):443-458.e7
Multiple system atrophy pathology is associated with primary Sjögren's syndrome.
Conway KS, Camelo-Piragua S, Fisher-Hubbard A, Perry WR, Shakkottai VG, Venneti S
JCI insight 2020 Aug 6;5(15)
JCI insight 2020 Aug 6;5(15)
Inhibition of DNAJ-HSP70 interaction improves strength in muscular dystrophy.
Bengoechea R, Findlay AR, Bhadra AK, Shao H, Stein KC, Pittman SK, Daw JA, Gestwicki JE, True HL, Weihl CC
The Journal of clinical investigation 2020 Aug 3;130(8):4470-4485
The Journal of clinical investigation 2020 Aug 3;130(8):4470-4485
Interactome Mapping Provides a Network of Neurodegenerative Disease Proteins and Uncovers Widespread Protein Aggregation in Affected Brains.
Haenig C, Atias N, Taylor AK, Mazza A, Schaefer MH, Russ J, Riechers SP, Jain S, Coughlin M, Fontaine JF, Freibaum BD, Brusendorf L, Zenkner M, Porras P, Stroedicke M, Schnoegl S, Arnsburg K, Boeddrich A, Pigazzini L, Heutink P, Taylor JP, Kirstein J, Andrade-Navarro MA, Sharan R, Wanker EE
Cell reports 2020 Aug 18;32(7):108050
Cell reports 2020 Aug 18;32(7):108050
Reprogramming fibroblasts and peripheral blood cells from a C9ORF72 patient: A proof-of-principle study.
Bardelli D, Sassone F, Colombrita C, Volpe C, Gumina V, Peverelli S, Catusi I, Ratti A, Silani V, Bossolasco P
Journal of cellular and molecular medicine 2020 Apr;24(7):4051-4060
Journal of cellular and molecular medicine 2020 Apr;24(7):4051-4060
Upregulation of ATG7 attenuates motor neuron dysfunction associated with depletion of TARDBP/TDP-43.
Donde A, Sun M, Jeong YH, Wen X, Ling J, Lin S, Braunstein K, Nie S, Wang S, Chen L, Wong PC
Autophagy 2020 Apr;16(4):672-682
Autophagy 2020 Apr;16(4):672-682
The Amyloid Inhibitor CLR01 Relieves Autophagy and Ameliorates Neuropathology in a Severe Lysosomal Storage Disease.
Monaco A, Maffia V, Sorrentino NC, Sambri I, Ezhova Y, Giuliano T, Cacace V, Nusco E, De Risi M, De Leonibus E, Schrader T, Klärner FG, Bitan G, Fraldi A
Molecular therapy : the journal of the American Society of Gene Therapy 2020 Apr 8;28(4):1167-1176
Molecular therapy : the journal of the American Society of Gene Therapy 2020 Apr 8;28(4):1167-1176
Astrocytes with TDP-43 inclusions exhibit reduced noradrenergic cAMP and Ca(2+) signaling and dysregulated cell metabolism.
Velebit J, Horvat A, Smolič T, Prpar Mihevc S, Rogelj B, Zorec R, Vardjan N
Scientific reports 2020 Apr 7;10(1):6003
Scientific reports 2020 Apr 7;10(1):6003
The cellular expression and proteolytic processing of the amyloid precursor protein is independent of TDP-43.
Hicks DA, Jones AC, Pickering-Brown SM, Hooper NM
Bioscience reports 2020 Apr 30;40(4)
Bioscience reports 2020 Apr 30;40(4)
Distinct molecular patterns of TDP-43 pathology in Alzheimer's disease: relationship with clinical phenotypes.
Tomé SO, Vandenberghe R, Ospitalieri S, Van Schoor E, Tousseyn T, Otto M, von Arnim CAF, Thal DR
Acta neuropathologica communications 2020 Apr 29;8(1):61
Acta neuropathologica communications 2020 Apr 29;8(1):61
Cell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD.
Khosravi B, LaClair KD, Riemenschneider H, Zhou Q, Frottin F, Mareljic N, Czuppa M, Farny D, Hartmann H, Michaelsen M, Arzberger T, Hartl FU, Hipp MS, Edbauer D
The EMBO journal 2020 Apr 15;39(8):e102811
The EMBO journal 2020 Apr 15;39(8):e102811
Fabry Disease With Concomitant Lewy Body Disease.
Del Tredici K, Ludolph AC, Feldengut S, Jacob C, Reichmann H, Bohl JR, Braak H
Journal of neuropathology and experimental neurology 2020 Apr 1;79(4):378-392
Journal of neuropathology and experimental neurology 2020 Apr 1;79(4):378-392
Characteristic Features of FUS Inclusions in Spinal Motor Neurons of Sporadic Amyotrophic Lateral Sclerosis.
Ikenaka K, Ishigaki S, Iguchi Y, Kawai K, Fujioka Y, Yokoi S, Abdelhamid RF, Nagano S, Mochizuki H, Katsuno M, Sobue G
Journal of neuropathology and experimental neurology 2020 Apr 1;79(4):370-377
Journal of neuropathology and experimental neurology 2020 Apr 1;79(4):370-377
Inactive USP14 and inactive UCHL5 cause accumulation of distinct ubiquitinated proteins in mammalian cells.
Chadchankar J, Korboukh V, Conway LC, Wobst HJ, Walker CA, Doig P, Jacobsen SJ, Brandon NJ, Moss SJ, Wang Q
PloS one 2019;14(11):e0225145
PloS one 2019;14(11):e0225145
Heterogeneous Nuclear Ribonucleoprotein E2 (hnRNP E2) Is a Component of TDP-43 Aggregates Specifically in the A and C Pathological Subtypes of Frontotemporal Lobar Degeneration.
Kattuah W, Rogelj B, King A, Shaw CE, Hortobágyi T, Troakes C
Frontiers in neuroscience 2019;13:551
Frontiers in neuroscience 2019;13:551
Developmental Expression of Mutant PFN1 in Motor Neurons Impacts Neuronal Growth and Motor Performance of Young and Adult Mice.
Brettle M, Stefen H, Djordjevic A, Fok SYY, Chan JW, van Hummel A, van der Hoven J, Przybyla M, Volkerling A, Ke YD, Delerue F, Ittner LM, Fath T
Frontiers in molecular neuroscience 2019;12:231
Frontiers in molecular neuroscience 2019;12:231
Repeated Low-Level Blast Overpressure Leads to Endovascular Disruption and Alterations in TDP-43 and Piezo2 in a Rat Model of Blast TBI.
Heyburn L, Abutarboush R, Goodrich S, Urioste R, Batuure A, Statz J, Wilder D, Ahlers ST, Long JB, Sajja VSSS
Frontiers in neurology 2019;10:766
Frontiers in neurology 2019;10:766
RNA Granules Hitchhike on Lysosomes for Long-Distance Transport, Using Annexin A11 as a Molecular Tether.
Liao YC, Fernandopulle MS, Wang G, Choi H, Hao L, Drerup CM, Patel R, Qamar S, Nixon-Abell J, Shen Y, Meadows W, Vendruscolo M, Knowles TPJ, Nelson M, Czekalska MA, Musteikyte G, Gachechiladze MA, Stephens CA, Pasolli HA, Forrest LR, St George-Hyslop P, Lippincott-Schwartz J, Ward ME
Cell 2019 Sep 19;179(1):147-164.e20
Cell 2019 Sep 19;179(1):147-164.e20
Myelin degeneration induced by mutant superoxide dismutase 1 accumulation promotes amyotrophic lateral sclerosis.
Kim S, Chung AY, Na JE, Lee SJ, Jeong SH, Kim E, Sun W, Rhyu IJ, Park HC
Glia 2019 Oct;67(10):1910-1921
Glia 2019 Oct;67(10):1910-1921
Expanded polyglutamine impairs normal nuclear distribution of fused in sarcoma and poly (rC)-binding protein 1 in Huntington's disease.
Mori S, Honda H, Ishii T, Yoshimura M, Sasagasako N, Suzuki SO, Taniwaki T, Iwaki T
Neuropathology : official journal of the Japanese Society of Neuropathology 2019 Oct;39(5):358-367
Neuropathology : official journal of the Japanese Society of Neuropathology 2019 Oct;39(5):358-367
TDP-43 regulates transcription at protein-coding genes and Alu retrotransposons.
Morera AA, Ahmed NS, Schwartz JC
Biochimica et biophysica acta. Gene regulatory mechanisms 2019 Oct;1862(10):194434
Biochimica et biophysica acta. Gene regulatory mechanisms 2019 Oct;1862(10):194434
Non-genetically modified models exhibit TARDBP mRNA increase due to perturbed TDP-43 autoregulation.
Sugai A, Kato T, Koyama A, Koike Y, Konno T, Ishihara T, Onodera O
Neurobiology of disease 2019 Oct;130:104534
Neurobiology of disease 2019 Oct;130:104534
Neuronal TDP-43 depletion affects activity-dependent plasticity.
Koza P, Beroun A, Konopka A, Górkiewicz T, Bijoch L, Torres JC, Bulska E, Knapska E, Kaczmarek L, Konopka W
Neurobiology of disease 2019 Oct;130:104499
Neurobiology of disease 2019 Oct;130:104499
Postmortem Cortex Samples Identify Distinct Molecular Subtypes of ALS: Retrotransposon Activation, Oxidative Stress, and Activated Glia.
Tam OH, Rozhkov NV, Shaw R, Kim D, Hubbard I, Fennessey S, Propp N, NYGC ALS Consortium, Fagegaltier D, Harris BT, Ostrow LW, Phatnani H, Ravits J, Dubnau J, Gale Hammell M
Cell reports 2019 Oct 29;29(5):1164-1177.e5
Cell reports 2019 Oct 29;29(5):1164-1177.e5
Phosphorylated TDP-43 aggregates in skeletal and cardiac muscle are a marker of myogenic degeneration in amyotrophic lateral sclerosis and various conditions.
Mori F, Tada M, Kon T, Miki Y, Tanji K, Kurotaki H, Tomiyama M, Ishihara T, Onodera O, Kakita A, Wakabayashi K
Acta neuropathologica communications 2019 Oct 28;7(1):165
Acta neuropathologica communications 2019 Oct 28;7(1):165
Phase separation-deficient TDP43 remains functional in splicing.
Schmidt HB, Barreau A, Rohatgi R
Nature communications 2019 Oct 25;10(1):4890
Nature communications 2019 Oct 25;10(1):4890
Design, synthesis, and biological evaluation of novel tetrahydroprotoberberine derivatives (THPBs) as proprotein convertase subtilisin/kexin type 9 (PCSK9) modulators for the treatment of hyperlipidemia.
Wu C, Xi C, Tong J, Zhao J, Jiang H, Wang J, Wang Y, Liu H
Acta pharmaceutica Sinica. B 2019 Nov;9(6):1216-1230
Acta pharmaceutica Sinica. B 2019 Nov;9(6):1216-1230
Neuropathological lesions in the very old: results from a large Brazilian autopsy study.
Suemoto CK, Leite REP, Ferretti-Rebustini REL, Rodriguez RD, Nitrini R, Pasqualucci CA, Jacob-Filho W, Grinberg LT
Brain pathology (Zurich, Switzerland) 2019 Nov;29(6):771-781
Brain pathology (Zurich, Switzerland) 2019 Nov;29(6):771-781
Clinicopathologic correlations in a family with a TBK1 mutation presenting as primary progressive aphasia and primary lateral sclerosis.
Hirsch-Reinshagen V, Alfaify OA, Hsiung GR, Pottier C, Baker M, Perkerson RB 3rd, Rademakers R, Briemberg H, Foti DJ, Mackenzie IR
Amyotrophic lateral sclerosis & frontotemporal degeneration 2019 Nov;20(7-8):568-575
Amyotrophic lateral sclerosis & frontotemporal degeneration 2019 Nov;20(7-8):568-575
Splicing repression is a major function of TDP-43 in motor neurons.
Donde A, Sun M, Ling JP, Braunstein KE, Pang B, Wen X, Cheng X, Chen L, Wong PC
Acta neuropathologica 2019 Nov;138(5):813-826
Acta neuropathologica 2019 Nov;138(5):813-826
Increased prevalence of granulovacuolar degeneration in C9orf72 mutation.
Riku Y, Duyckaerts C, Boluda S, Plu I, Le Ber I, Millecamps S, Salachas F, Brainbank NeuroCEB Neuropathology Network, Yoshida M, Ando T, Katsuno M, Sobue G, Seilhean D
Acta neuropathologica 2019 Nov;138(5):783-793
Acta neuropathologica 2019 Nov;138(5):783-793
Neuronal over-expression of Oxr1 is protective against ALS-associated mutant TDP-43 mislocalisation in motor neurons and neuromuscular defects in vivo.
Williamson MG, Finelli MJ, Sleigh JN, Reddington A, Gordon D, Talbot K, Davies KE, Oliver PL
Human molecular genetics 2019 Nov 1;28(21):3584-3599
Human molecular genetics 2019 Nov 1;28(21):3584-3599
Memory Decline and Its Reversal in Aging and Neurodegeneration Involve miR-183/96/182 Biogenesis.
Jawaid A, Woldemichael BT, Kremer EA, Laferriere F, Gaur N, Afroz T, Polymenidou M, Mansuy IM
Molecular neurobiology 2019 May;56(5):3451-3462
Molecular neurobiology 2019 May;56(5):3451-3462
Dysregulation of TDP-43 intracellular localization and early onset ALS are associated with a TARDBP S375G variant.
Newell K, Paron F, Mompean M, Murrell J, Salis E, Stuani C, Pattee G, Romano M, Laurents D, Ghetti B, Buratti E
Brain pathology (Zurich, Switzerland) 2019 May;29(3):397-413
Brain pathology (Zurich, Switzerland) 2019 May;29(3):397-413
TDP-43 induces mitochondrial damage and activates the mitochondrial unfolded protein response.
Wang P, Deng J, Dong J, Liu J, Bigio EH, Mesulam M, Wang T, Sun L, Wang L, Lee AY, McGee WA, Chen X, Fushimi K, Zhu L, Wu JY
PLoS genetics 2019 May;15(5):e1007947
PLoS genetics 2019 May;15(5):e1007947
Proteomic analysis reveals that wildtype and alanine-expanded nuclear poly(A)-binding protein exhibit differential interactions in skeletal muscle.
Banerjee A, Phillips BL, Deng Q, Seyfried NT, Pavlath GK, Vest KE, Corbett AH
The Journal of biological chemistry 2019 May 3;294(18):7360-7376
The Journal of biological chemistry 2019 May 3;294(18):7360-7376
ULK1 and ULK2 Regulate Stress Granule Disassembly Through Phosphorylation and Activation of VCP/p97.
Wang B, Maxwell BA, Joo JH, Gwon Y, Messing J, Mishra A, Shaw TI, Ward AL, Quan H, Sakurada SM, Pruett-Miller SM, Bertorini T, Vogel P, Kim HJ, Peng J, Taylor JP, Kundu M
Molecular cell 2019 May 16;74(4):742-757.e8
Molecular cell 2019 May 16;74(4):742-757.e8
Pathological Modification of TDP-43 in Amyotrophic Lateral Sclerosis with SOD1 Mutations.
Jeon GS, Shim YM, Lee DY, Kim JS, Kang M, Ahn SH, Shin JY, Geum D, Hong YH, Sung JJ
Molecular neurobiology 2019 Mar;56(3):2007-2021
Molecular neurobiology 2019 Mar;56(3):2007-2021
PARylation regulates stress granule dynamics, phase separation, and neurotoxicity of disease-related RNA-binding proteins.
Duan Y, Du A, Gu J, Duan G, Wang C, Gui X, Ma Z, Qian B, Deng X, Zhang K, Sun L, Tian K, Zhang Y, Jiang H, Liu C, Fang Y
Cell research 2019 Mar;29(3):233-247
Cell research 2019 Mar;29(3):233-247
TDP-43 levels are higher in platelets from patients with sporadic amyotrophic lateral sclerosis than in healthy controls.
Hishizawa M, Yamashita H, Akizuki M, Urushitani M, Takahashi R
Neurochemistry international 2019 Mar;124:41-45
Neurochemistry international 2019 Mar;124:41-45
Aggregation of the nucleic acid-binding protein TDP-43 occurs via distinct routes that are coordinated with stress granule formation.
Chen Y, Cohen TJ
The Journal of biological chemistry 2019 Mar 8;294(10):3696-3706
The Journal of biological chemistry 2019 Mar 8;294(10):3696-3706
Structure of Calcarisporiella thermophila Hsp104 Disaggregase that Antagonizes Diverse Proteotoxic Misfolding Events.
Michalska K, Zhang K, March ZM, Hatzos-Skintges C, Pintilie G, Bigelow L, Castellano LM, Miles LJ, Jackrel ME, Chuang E, Jedrzejczak R, Shorter J, Chiu W, Joachimiak A
Structure (London, England : 1993) 2019 Mar 5;27(3):449-463.e7
Structure (London, England : 1993) 2019 Mar 5;27(3):449-463.e7
Biomarkers for diseases with TDP-43 pathology.
Steinacker P, Barschke P, Otto M
Molecular and cellular neurosciences 2019 Jun;97:43-59
Molecular and cellular neurosciences 2019 Jun;97:43-59
Toxic expanded GGGGCC repeat transcription is mediated by the PAF1 complex in C9orf72-associated FTD.
Goodman LD, Prudencio M, Kramer NJ, Martinez-Ramirez LF, Srinivasan AR, Lan M, Parisi MJ, Zhu Y, Chew J, Cook CN, Berson A, Gitler AD, Petrucelli L, Bonini NM
Nature neuroscience 2019 Jun;22(6):863-874
Nature neuroscience 2019 Jun;22(6):863-874
VCP maintains lysosomal homeostasis and TFEB activity in differentiated skeletal muscle.
Arhzaouy K, Papadopoulos C, Schulze N, Pittman SK, Meyer H, Weihl CC
Autophagy 2019 Jun;15(6):1082-1099
Autophagy 2019 Jun;15(6):1082-1099
Cross-Regulation between TDP-43 and Paraspeckles Promotes Pluripotency-Differentiation Transition.
Modic M, Grosch M, Rot G, Schirge S, Lepko T, Yamazaki T, Lee FCY, Rusha E, Shaposhnikov D, Palo M, Merl-Pham J, Cacchiarelli D, Rogelj B, Hauck SM, von Mering C, Meissner A, Lickert H, Hirose T, Ule J, Drukker M
Molecular cell 2019 Jun 6;74(5):951-965.e13
Molecular cell 2019 Jun 6;74(5):951-965.e13
Association between TDP-43 and mitochondria in inclusion body myositis.
Huntley ML, Gao J, Termsarasab P, Wang L, Zeng S, Thammongkolchai T, Liu Y, Cohen ML, Wang X
Laboratory investigation; a journal of technical methods and pathology 2019 Jul;99(7):1041-1048
Laboratory investigation; a journal of technical methods and pathology 2019 Jul;99(7):1041-1048
Autosomal dominant mitochondrial membrane protein-associated neurodegeneration (MPAN).
Gregory A, Lotia M, Jeong SY, Fox R, Zhen D, Sanford L, Hamada J, Jahic A, Beetz C, Freed A, Kurian MA, Cullup T, van der Weijden MCM, Nguyen V, Setthavongsack N, Garcia D, Krajbich V, Pham T, Woltjer R, George BP, Minks KQ, Paciorkowski AR, Hogarth P, Jankovic J, Hayflick SJ
Molecular genetics & genomic medicine 2019 Jul;7(7):e00736
Molecular genetics & genomic medicine 2019 Jul;7(7):e00736
Neuronal Expression of UBQLN2(P497H) Exacerbates TDP-43 Pathology in TDP-43(G348C) Mice through Interaction with Ubiquitin.
Picher-Martel V, Renaud L, Bareil C, Julien JP
Molecular neurobiology 2019 Jul;56(7):4680-4696
Molecular neurobiology 2019 Jul;56(7):4680-4696
TDP-43 regulates early-phase insulin secretion via CaV1.2-mediated exocytosis in islets.
Araki K, Araki A, Honda D, Izumoto T, Hashizume A, Hijikata Y, Yamada S, Iguchi Y, Hara A, Ikumi K, Kawai K, Ishigaki S, Nakamichi Y, Tsunekawa S, Seino Y, Yamamoto A, Takayama Y, Hidaka S, Tominaga M, Ohara-Imaizumi M, Suzuki A, Ishiguro H, Enomoto A, Yoshida M, Arima H, Muramatsu SI, Sobue G, Katsuno M
The Journal of clinical investigation 2019 Jul 29;129(9):3578-3593
The Journal of clinical investigation 2019 Jul 29;129(9):3578-3593
C-terminal and full length TDP-43 specie differ according to FTLD-TDP lesion type but not genetic mutation.
Josephs KA, Zhang YJ, Baker M, Rademakers R, Petrucelli L, Dickson DW
Acta neuropathologica communications 2019 Jul 2;7(1):100
Acta neuropathologica communications 2019 Jul 2;7(1):100
Motor dysfunction and neurodegeneration in a C9orf72 mouse line expressing poly-PR.
Hao Z, Liu L, Tao Z, Wang R, Ren H, Sun H, Lin Z, Zhang Z, Mu C, Zhou J, Wang G
Nature communications 2019 Jul 2;10(1):2906
Nature communications 2019 Jul 2;10(1):2906
Identification and therapeutic rescue of autophagosome and glutamate receptor defects in C9ORF72 and sporadic ALS neurons.
Shi Y, Hung ST, Rocha G, Lin S, Linares GR, Staats KA, Seah C, Wang Y, Chickering M, Lai J, Sugawara T, Sagare AP, Zlokovic BV, Ichida JK
JCI insight 2019 Jul 16;5(15)
JCI insight 2019 Jul 16;5(15)
Persistent neuropathological effects 14 years following amyloid-β immunization in Alzheimer's disease.
Nicoll JAR, Buckland GR, Harrison CH, Page A, Harris S, Love S, Neal JW, Holmes C, Boche D
Brain : a journal of neurology 2019 Jul 1;142(7):2113-2126
Brain : a journal of neurology 2019 Jul 1;142(7):2113-2126
Impaired Cu-Zn Superoxide Dismutase (SOD1) and Calcineurin (Cn) Interaction in ALS: A Presumed Consequence for TDP-43 and Zinc Aggregation in Tg SOD1(G93A) Rodent Spinal Cord Tissue.
Kim JM, Billington E, Reyes A, Notarianni T, Sage J, Agbas E, Taylor M, Monast I, Stanford JA, Agbas A
Neurochemical research 2019 Jan;44(1):228-233
Neurochemical research 2019 Jan;44(1):228-233
Development of an automated capillary nano-immunoassay-Simple Western assay-to quantify total TDP43 protein in human platelet samples.
Fourier A, Escal J, Bernard E, Lachman I, Perret-Liaudet A, Leblanc P, Quadrio I
Analytical and bioanalytical chemistry 2019 Jan;411(1):267-275
Analytical and bioanalytical chemistry 2019 Jan;411(1):267-275
TDP-43 extracted from frontotemporal lobar degeneration subject brains displays distinct aggregate assemblies and neurotoxic effects reflecting disease progression rates.
Laferrière F, Maniecka Z, Pérez-Berlanga M, Hruska-Plochan M, Gilhespy L, Hock EM, Wagner U, Afroz T, Boersema PJ, Barmettler G, Foti SC, Asi YT, Isaacs AM, Al-Amoudi A, Lewis A, Stahlberg H, Ravits J, De Giorgi F, Ichas F, Bezard E, Picotti P, Lashley T, Polymenidou M
Nature neuroscience 2019 Jan;22(1):65-77
Nature neuroscience 2019 Jan;22(1):65-77
Impaired Nucleoporins Are Present in Sporadic Amyotrophic Lateral Sclerosis Motor Neurons that Exhibit Mislocalization of the 43-kDa TAR DNA-Binding Protein.
Aizawa H, Yamashita T, Kato H, Kimura T, Kwak S
Journal of clinical neurology (Seoul, Korea) 2019 Jan;15(1):62-67
Journal of clinical neurology (Seoul, Korea) 2019 Jan;15(1):62-67
Neurons selectively targeted in frontotemporal dementia reveal early stage TDP-43 pathobiology.
Nana AL, Sidhu M, Gaus SE, Hwang JL, Li L, Park Y, Kim EJ, Pasquini L, Allen IE, Rankin KP, Toller G, Kramer JH, Geschwind DH, Coppola G, Huang EJ, Grinberg LT, Miller BL, Seeley WW
Acta neuropathologica 2019 Jan;137(1):27-46
Acta neuropathologica 2019 Jan;137(1):27-46
Single-copy expression of an amyotrophic lateral sclerosis-linked TDP-43 mutation (M337V) in BAC transgenic mice leads to altered stress granule dynamics and progressive motor dysfunction.
Gordon D, Dafinca R, Scaber J, Alegre-Abarrategui J, Farrimond L, Scott C, Biggs D, Kent L, Oliver PL, Davies B, Ansorge O, Wade-Martins R, Talbot K
Neurobiology of disease 2019 Jan;121:148-162
Neurobiology of disease 2019 Jan;121:148-162
Mutant TDP-43 Causes Early-Stage Dose-Dependent Motor Neuron Degeneration in a TARDBP Knockin Mouse Model of ALS.
Ebstein SY, Yagudayeva I, Shneider NA
Cell reports 2019 Jan 8;26(2):364-373.e4
Cell reports 2019 Jan 8;26(2):364-373.e4
(18)F-flortaucipir (AV-1451) tau PET in frontotemporal dementia syndromes.
Tsai RM, Bejanin A, Lesman-Segev O, LaJoie R, Visani A, Bourakova V, O'Neil JP, Janabi M, Baker S, Lee SE, Perry DC, Bajorek L, Karydas A, Spina S, Grinberg LT, Seeley WW, Ramos EM, Coppola G, Gorno-Tempini ML, Miller BL, Rosen HJ, Jagust W, Boxer AL, Rabinovici GD
Alzheimer's research & therapy 2019 Jan 31;11(1):13
Alzheimer's research & therapy 2019 Jan 31;11(1):13
Rasagiline for amyotrophic lateral sclerosis: A randomized, controlled trial.
Statland JM, Moore D, Wang Y, Walsh M, Mozaffar T, Elman L, Nations SP, Mitsumoto H, Fernandes JA, Saperstein D, Hayat G, Herbelin L, Karam C, Katz J, Wilkins HM, Agbas A, Swerdlow RH, Santella RM, Dimachkie MM, Barohn RJ, Rasagiline Investigators of the Muscle Study Group and Western ALS Consortium
Muscle & nerve 2019 Feb;59(2):201-207
Muscle & nerve 2019 Feb;59(2):201-207
Premature polyadenylation-mediated loss of stathmin-2 is a hallmark of TDP-43-dependent neurodegeneration.
Melamed Z, López-Erauskin J, Baughn MW, Zhang O, Drenner K, Sun Y, Freyermuth F, McMahon MA, Beccari MS, Artates JW, Ohkubo T, Rodriguez M, Lin N, Wu D, Bennett CF, Rigo F, Da Cruz S, Ravits J, Lagier-Tourenne C, Cleveland DW
Nature neuroscience 2019 Feb;22(2):180-190
Nature neuroscience 2019 Feb;22(2):180-190
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair.
Klim JR, Williams LA, Limone F, Guerra San Juan I, Davis-Dusenbery BN, Mordes DA, Burberry A, Steinbaugh MJ, Gamage KK, Kirchner R, Moccia R, Cassel SH, Chen K, Wainger BJ, Woolf CJ, Eggan K
Nature neuroscience 2019 Feb;22(2):167-179
Nature neuroscience 2019 Feb;22(2):167-179
Brain banking in low and middle-income countries: Raison D'être for the Ibadan Brain Ageing, Dementia And Neurodegeneration (IBADAN) Brain Bank Project.
Akinyemi RO, Salami A, Akinyemi J, Ojagbemi A, Olopade F, Coker M, Farombi T, Nweke M, Arulogun O, Jegede A, Owolabi M, Kalaria RN, Ogunniyi A
Brain research bulletin 2019 Feb;145:136-141
Brain research bulletin 2019 Feb;145:136-141
Corticobasal syndrome with visual hallucinations and probable REM-sleep behavior disorder: an autopsied case report of a patient with CBD and LBD pathology.
Naasan G, Shany-Ur T, Sidhu M, Barton C, Ketelle R, Shdo SM, Kramer JH, Miller BL, Seeley WW
Neurocase 2019 Feb-Apr;25(1-2):26-33
Neurocase 2019 Feb-Apr;25(1-2):26-33
Virus-mediated delivery of antibody targeting TAR DNA-binding protein-43 mitigates associated neuropathology.
Pozzi S, Thammisetty SS, Codron P, Rahimian R, Plourde KV, Soucy G, Bareil C, Phaneuf D, Kriz J, Gravel C, Julien JP
The Journal of clinical investigation 2019 Feb 25;129(4):1581-1595
The Journal of clinical investigation 2019 Feb 25;129(4):1581-1595
The Small Non-coding Vault RNA1-1 Acts as a Riboregulator of Autophagy.
Horos R, Büscher M, Kleinendorst R, Alleaume AM, Tarafder AK, Schwarzl T, Dziuba D, Tischer C, Zielonka EM, Adak A, Castello A, Huber W, Sachse C, Hentze MW
Cell 2019 Feb 21;176(5):1054-1067.e12
Cell 2019 Feb 21;176(5):1054-1067.e12
Overriding FUS autoregulation in mice triggers gain-of-toxic dysfunctions in RNA metabolism and autophagy-lysosome axis.
Ling SC, Dastidar SG, Tokunaga S, Ho WY, Lim K, Ilieva H, Parone PA, Tyan SH, Tse TM, Chang JC, Platoshyn O, Bui NB, Bui A, Vetto A, Sun S, McAlonis-Downes M, Han JS, Swing D, Kapeli K, Yeo GW, Tessarollo L, Marsala M, Shaw CE, Tucker-Kellogg G, La Spada AR, Lagier-Tourenne C, Da Cruz S, Cleveland DW
eLife 2019 Feb 12;8
eLife 2019 Feb 12;8
Physiologically Important Electrolytes as Regulators of TDP-43 Aggregation and Droplet-Phase Behavior.
Sun Y, Medina Cruz A, Hadley KC, Galant NJ, Law R, Vernon RM, Morris VK, Robertson J, Chakrabartty A
Biochemistry 2019 Feb 12;58(6):590-607
Biochemistry 2019 Feb 12;58(6):590-607
Muscle pathology of hereditary motor and sensory neuropathy with proximal dominant involvement with TFG mutation.
Yamashita S, Kimura E, Zhang Z, Tawara N, Hara K, Yoshimura A, Takashima H, Ando Y
Muscle & nerve 2019 Dec;60(6):739-744
Muscle & nerve 2019 Dec;60(6):739-744
The long noncoding RNA neuroLNC regulates presynaptic activity by interacting with the neurodegeneration-associated protein TDP-43.
Keihani S, Kluever V, Mandad S, Bansal V, Rahman R, Fritsch E, Gomes LC, Gärtner A, Kügler S, Urlaub H, Wren JD, Bonn S, Rizzoli SO, Fornasiero EF
Science advances 2019 Dec;5(12):eaay2670
Science advances 2019 Dec;5(12):eaay2670
Neuroglial patterns are shared by cerebella from prion and prion-like disorder affected patients.
Garcés M, Guijarro MI, Vargas A, Badiola JJ, Monzón M
Mechanisms of ageing and development 2019 Dec;184:111176
Mechanisms of ageing and development 2019 Dec;184:111176
TDP-43 knockdown causes innate immune activation via protein kinase R in astrocytes.
LaRocca TJ, Mariani A, Watkins LR, Link CD
Neurobiology of disease 2019 Dec;132:104514
Neurobiology of disease 2019 Dec;132:104514
Luminescent conjugated oligothiophenes distinguish between α-synuclein assemblies of Parkinson's disease and multiple system atrophy.
Klingstedt T, Ghetti B, Holton JL, Ling H, Nilsson KPR, Goedert M
Acta neuropathologica communications 2019 Dec 3;7(1):193
Acta neuropathologica communications 2019 Dec 3;7(1):193
Activity of the poly(A) binding protein MSUT2 determines susceptibility to pathological tau in the mammalian brain.
Wheeler JM, McMillan P, Strovas TJ, Liachko NF, Amlie-Wolf A, Kow RL, Klein RL, Szot P, Robinson L, Guthrie C, Saxton A, Kanaan NM, Raskind M, Peskind E, Trojanowski JQ, Lee VMY, Wang LS, Keene CD, Bird T, Schellenberg GD, Kraemer B
Science translational medicine 2019 Dec 18;11(523)
Science translational medicine 2019 Dec 18;11(523)
Nopp140-mediated concentration of telomerase in Cajal bodies regulates telomere length.
Bizarro J, Bhardwaj A, Smith S, Meier UT
Molecular biology of the cell 2019 Dec 15;30(26):3136-3150
Molecular biology of the cell 2019 Dec 15;30(26):3136-3150
Compromised function of the ESCRT pathway promotes endolysosomal escape of tau seeds and propagation of tau aggregation.
Chen JJ, Nathaniel DL, Raghavan P, Nelson M, Tian R, Tse E, Hong JY, See SK, Mok SA, Hein MY, Southworth DR, Grinberg LT, Gestwicki JE, Leonetti MD, Kampmann M
The Journal of biological chemistry 2019 Dec 13;294(50):18952-18966
The Journal of biological chemistry 2019 Dec 13;294(50):18952-18966
Suppression of Progranulin Expression Leads to Formation of Intranuclear TDP-43 Inclusions In Vitro: A Cell Model of Frontotemporal Lobar Degeneration.
Zhu J, Wang N, Li X, Zheng X, Zhao J, Xia H, Mao Q
Journal of neuropathology and experimental neurology 2019 Dec 1;78(12):1124-1129
Journal of neuropathology and experimental neurology 2019 Dec 1;78(12):1124-1129
RRM adjacent TARDBP mutations disrupt RNA binding and enhance TDP-43 proteinopathy.
Chen HJ, Topp SD, Hui HS, Zacco E, Katarya M, McLoughlin C, King A, Smith BN, Troakes C, Pastore A, Shaw CE
Brain : a journal of neurology 2019 Dec 1;142(12):3753-3770
Brain : a journal of neurology 2019 Dec 1;142(12):3753-3770
Altered immunoreactivity of ErbB4, a causative gene product for ALS19, in the spinal cord of patients with sporadic ALS.
Takahashi Y, Uchino A, Shioya A, Sano T, Matsumoto C, Numata-Uematsu Y, Nagano S, Araki T, Murayama S, Saito Y
Neuropathology : official journal of the Japanese Society of Neuropathology 2019 Aug;39(4):268-278
Neuropathology : official journal of the Japanese Society of Neuropathology 2019 Aug;39(4):268-278
Mining Disaggregase Sequence Space to Safely Counter TDP-43, FUS, and α-Synuclein Proteotoxicity.
Tariq A, Lin J, Jackrel ME, Hesketh CD, Carman PJ, Mack KL, Weitzman R, Gambogi C, Hernandez Murillo OA, Sweeny EA, Gurpinar E, Yokom AL, Gates SN, Yee K, Sudesh S, Stillman J, Rizo AN, Southworth DR, Shorter J
Cell reports 2019 Aug 20;28(8):2080-2095.e6
Cell reports 2019 Aug 20;28(8):2080-2095.e6
CYLD dysregulation in pathogenesis of sporadic inclusion body myositis.
Yamashita S, Matsuo Y, Tawara N, Hara K, Yamamoto M, Nishikami T, Kawakami K, Zhang X, Zhang Z, Doki T, Ando Y
Scientific reports 2019 Aug 12;9(1):11606
Scientific reports 2019 Aug 12;9(1):11606
Amyotrophic lateral sclerosis-associated TDP-43 mutation Q331K prevents nuclear translocation of XRCC4-DNA ligase 4 complex and is linked to genome damage-mediated neuronal apoptosis.
Guerrero EN, Mitra J, Wang H, Rangaswamy S, Hegde PM, Basu P, Rao KS, Hegde ML
Human molecular genetics 2019 Aug 1;28(15):2459-2476
Human molecular genetics 2019 Aug 1;28(15):2459-2476
Recapitulation of Pathological TDP-43 Features in Immortalized Lymphocytes from Sporadic ALS Patients.
Posa D, Martínez-González L, Bartolomé F, Nagaraj S, Porras G, Martínez A, Martín-Requero Á
Molecular neurobiology 2019 Apr;56(4):2424-2432
Molecular neurobiology 2019 Apr;56(4):2424-2432
Motor neuron degeneration, severe myopathy and TDP-43 increase in a transgenic pig model of SOD1-linked familiar ALS.
Crociara P, Chieppa MN, Vallino Costassa E, Berrone E, Gallo M, Lo Faro M, Pintore MD, Iulini B, D'Angelo A, Perona G, Botter A, Formicola D, Rainoldi A, Paulis M, Vezzoni P, Meli F, Peverali FA, Bendotti C, Trolese MC, Pasetto L, Bonetto V, Lazzari G, Duchi R, Perota A, Lagutina I, Quadalti C, Gennero MS, Dezzutto D, Desiato R, Boido M, Ghibaudi M, Valentini MC, Caramelli M, Galli C, Casalone C, Corona C
Neurobiology of disease 2019 Apr;124:263-275
Neurobiology of disease 2019 Apr;124:263-275
Loss of Nuclear TDP-43 Is Associated with Decondensation of LINE Retrotransposons.
Liu EY, Russ J, Cali CP, Phan JM, Amlie-Wolf A, Lee EB
Cell reports 2019 Apr 30;27(5):1409-1421.e6
Cell reports 2019 Apr 30;27(5):1409-1421.e6
Deletion of Tbk1 disrupts autophagy and reproduces behavioral and locomotor symptoms of FTD-ALS in mice.
Duan W, Guo M, Yi L, Zhang J, Bi Y, Liu Y, Li Y, Li Z, Ma Y, Zhang G, Liu Y, Song X, Li C
Aging 2019 Apr 30;11(8):2457-2476
Aging 2019 Apr 30;11(8):2457-2476
Drosophila Ref1/ALYREF regulates transcription and toxicity associated with ALS/FTD disease etiologies.
Berson A, Goodman LD, Sartoris AN, Otte CG, Aykit JA, Lee VM, Trojanowski JQ, Bonini NM
Acta neuropathologica communications 2019 Apr 29;7(1):65
Acta neuropathologica communications 2019 Apr 29;7(1):65
Detection of TAR DNA-binding protein 43 (TDP-43) oligomers as initial intermediate species during aggregate formation.
French RL, Grese ZR, Aligireddy H, Dhavale DD, Reeb AN, Kedia N, Kotzbauer PT, Bieschke J, Ayala YM
The Journal of biological chemistry 2019 Apr 26;294(17):6696-6709
The Journal of biological chemistry 2019 Apr 26;294(17):6696-6709
Genetic Regulation of Neuronal Progranulin Reveals a Critical Role for the Autophagy-Lysosome Pathway.
Elia LP, Mason AR, Alijagic A, Finkbeiner S
The Journal of neuroscience : the official journal of the Society for Neuroscience 2019 Apr 24;39(17):3332-3344
The Journal of neuroscience : the official journal of the Society for Neuroscience 2019 Apr 24;39(17):3332-3344
Cytoplasmic TDP-43 De-mixing Independent of Stress Granules Drives Inhibition of Nuclear Import, Loss of Nuclear TDP-43, and Cell Death.
Gasset-Rosa F, Lu S, Yu H, Chen C, Melamed Z, Guo L, Shorter J, Da Cruz S, Cleveland DW
Neuron 2019 Apr 17;102(2):339-357.e7
Neuron 2019 Apr 17;102(2):339-357.e7
RNA Binding Antagonizes Neurotoxic Phase Transitions of TDP-43.
Mann JR, Gleixner AM, Mauna JC, Gomes E, DeChellis-Marks MR, Needham PG, Copley KE, Hurtle B, Portz B, Pyles NJ, Guo L, Calder CB, Wills ZP, Pandey UB, Kofler JK, Brodsky JL, Thathiah A, Shorter J, Donnelly CJ
Neuron 2019 Apr 17;102(2):321-338.e8
Neuron 2019 Apr 17;102(2):321-338.e8
Properties of LINE-1 proteins and repeat element expression in the context of amyotrophic lateral sclerosis.
Pereira GC, Sanchez L, Schaughency PM, Rubio-Roldán A, Choi JA, Planet E, Batra R, Turelli P, Trono D, Ostrow LW, Ravits J, Kazazian HH, Wheelan SJ, Heras SR, Mayer J, García-Pérez JL, Goodier JL
Mobile DNA 2018;9:35
Mobile DNA 2018;9:35
Myopathy With SQSTM1 and TIA1 Variants: Clinical and Pathological Features.
Niu Z, Pontifex CS, Berini S, Hamilton LE, Naddaf E, Wieben E, Aleff RA, Martens K, Gruber A, Engel AG, Pfeffer G, Milone M
Frontiers in neurology 2018;9:147
Frontiers in neurology 2018;9:147
Methods to Investigate the Molecular Basis of Progranulin Actions on Brain and Behavior In Vivo Using Knockout Mice.
Boddaert J, Wils H, Kumar-Singh S
Methods in molecular biology (Clifton, N.J.) 2018;1806:233-253
Methods in molecular biology (Clifton, N.J.) 2018;1806:233-253
Cryptic exon splicing function of TARDBP interacts with autophagy in nervous tissue.
Torres P, Ramírez-Núñez O, Romero-Guevara R, Barés G, Granado-Serrano AB, Ayala V, Boada J, Fontdevila L, Povedano M, Sanchís D, Pamplona R, Ferrer I, Portero-Otín M
Autophagy 2018;14(8):1398-1403
Autophagy 2018;14(8):1398-1403
mTh1 driven expression of hTDP-43 results in typical ALS/FTLD neuropathological symptoms.
Scherz B, Rabl R, Flunkert S, Rohler S, Neddens J, Taub N, Temmel M, Panzenboeck U, Niederkofler V, Zimmermann R, Hutter-Paier B
PloS one 2018;13(5):e0197674
PloS one 2018;13(5):e0197674
DGCR8 Promotes Neural Progenitor Expansion and Represses Neurogenesis in the Mouse Embryonic Neocortex.
Hoffmann N, Weise SC, Marinaro F, Vogel T, De Pietri Tonelli D
Frontiers in neuroscience 2018;12:281
Frontiers in neuroscience 2018;12:281
Staufen1 links RNA stress granules and autophagy in a model of neurodegeneration.
Paul S, Dansithong W, Figueroa KP, Scoles DR, Pulst SM
Nature communications 2018 Sep 7;9(1):3648
Nature communications 2018 Sep 7;9(1):3648
Poly(ADP-Ribose) Prevents Pathological Phase Separation of TDP-43 by Promoting Liquid Demixing and Stress Granule Localization.
McGurk L, Gomes E, Guo L, Mojsilovic-Petrovic J, Tran V, Kalb RG, Shorter J, Bonini NM
Molecular cell 2018 Sep 6;71(5):703-717.e9
Molecular cell 2018 Sep 6;71(5):703-717.e9
TBK1 Suppresses RIPK1-Driven Apoptosis and Inflammation during Development and in Aging.
Xu D, Jin T, Zhu H, Chen H, Ofengeim D, Zou C, Mifflin L, Pan L, Amin P, Li W, Shan B, Naito MG, Meng H, Li Y, Pan H, Aron L, Adiconis X, Levin JZ, Yankner BA, Yuan J
Cell 2018 Sep 6;174(6):1477-1491.e19
Cell 2018 Sep 6;174(6):1477-1491.e19
Distinct multilevel misregulations of Parkin and PINK1 revealed in cell and animal models of TDP-43 proteinopathy.
Sun X, Duan Y, Qin C, Li JC, Duan G, Deng X, Ni J, Cao X, Xiang K, Tian K, Chen CH, Li A, Fang Y
Cell death & disease 2018 Sep 20;9(10):953
Cell death & disease 2018 Sep 20;9(10):953
FTLD/ALS-linked TDP-43 mutations do not alter TDP-43's ability to self-regulate its expression in Drosophila.
Miguel L, Avequin T, Pons M, Frebourg T, Campion D, Lecourtois M
Brain research 2018 Sep 15;1695:1-9
Brain research 2018 Sep 15;1695:1-9
Spreading of Tau Pathology in Sporadic Alzheimer's Disease Along Cortico-cortical Top-Down Connections.
Braak H, Del Tredici K
Cerebral cortex (New York, N.Y. : 1991) 2018 Sep 1;28(9):3372-3384
Cerebral cortex (New York, N.Y. : 1991) 2018 Sep 1;28(9):3372-3384
Towards a TDP-43-Based Biomarker for ALS and FTLD.
Feneberg E, Gray E, Ansorge O, Talbot K, Turner MR
Molecular neurobiology 2018 Oct;55(10):7789-7801
Molecular neurobiology 2018 Oct;55(10):7789-7801
Colocalization of Bunina bodies and TDP-43 inclusions in a case of sporadic amyotrophic lateral sclerosis with Lewy body-like hyaline inclusions.
Miki Y, Mori F, Seino Y, Tanji K, Yoshizawa T, Kijima H, Shoji M, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2018 Oct;38(5):521-528
Neuropathology : official journal of the Japanese Society of Neuropathology 2018 Oct;38(5):521-528
Subarachnoid hemorrhage enhances the expression of TDP-43 in the brain of experimental rats and human subjects.
He T, Zuo Y, Ai-Zakwani K, Luo J, Zhu H, Yan XX, Liu F
Experimental and therapeutic medicine 2018 Oct;16(4):3363-3368
Experimental and therapeutic medicine 2018 Oct;16(4):3363-3368
Mutant UBQLN2 promotes toxicity by modulating intrinsic self-assembly.
Sharkey LM, Safren N, Pithadia AS, Gerson JE, Dulchavsky M, Fischer S, Patel R, Lantis G, Ashraf N, Kim JH, Meliki A, Minakawa EN, Barmada SJ, Ivanova MI, Paulson HL
Proceedings of the National Academy of Sciences of the United States of America 2018 Oct 30;115(44):E10495-E10504
Proceedings of the National Academy of Sciences of the United States of America 2018 Oct 30;115(44):E10495-E10504
Aberrant activation of non-coding RNA targets of transcriptional elongation complexes contributes to TDP-43 toxicity.
Chung CY, Berson A, Kennerdell JR, Sartoris A, Unger T, Porta S, Kim HJ, Smith ER, Shilatifard A, Van Deerlin V, Lee VM, Chen-Plotkin A, Bonini NM
Nature communications 2018 Oct 23;9(1):4406
Nature communications 2018 Oct 23;9(1):4406
Progranulin reduces insoluble TDP-43 levels, slows down axonal degeneration and prolongs survival in mutant TDP-43 mice.
Beel S, Herdewyn S, Fazal R, De Decker M, Moisse M, Robberecht W, Van Den Bosch L, Van Damme P
Molecular neurodegeneration 2018 Oct 16;13(1):55
Molecular neurodegeneration 2018 Oct 16;13(1):55
Epigenetic Changes Associated with the Expression of Amyotrophic Lateral Sclerosis (ALS) Causing Genes.
Masala A, Sanna S, Esposito S, Rassu M, Galioto M, Zinellu A, Carru C, Carrì MT, Iaccarino C, Crosio C
Neuroscience 2018 Oct 15;390:1-11
Neuroscience 2018 Oct 15;390:1-11
Identification and characterization of ubiquitinylation sites in TAR DNA-binding protein of 43 kDa (TDP-43).
Hans F, Eckert M, von Zweydorf F, Gloeckner CJ, Kahle PJ
The Journal of biological chemistry 2018 Oct 12;293(41):16083-16099
The Journal of biological chemistry 2018 Oct 12;293(41):16083-16099
TAR DNA-Binding Protein 43 and Disrupted in Schizophrenia 1 Coaggregation Disrupts Dendritic Local Translation and Mental Function in Frontotemporal Lobar Degeneration.
Endo R, Takashima N, Nekooki-Machida Y, Komi Y, Hui KK, Takao M, Akatsu H, Murayama S, Sawa A, Tanaka M
Biological psychiatry 2018 Oct 1;84(7):509-521
Biological psychiatry 2018 Oct 1;84(7):509-521
TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle.
Vogler TO, Wheeler JR, Nguyen ED, Hughes MP, Britson KA, Lester E, Rao B, Betta ND, Whitney ON, Ewachiw TE, Gomes E, Shorter J, Lloyd TE, Eisenberg DS, Taylor JP, Johnson AM, Olwin BB, Parker R
Nature 2018 Nov;563(7732):508-513
Nature 2018 Nov;563(7732):508-513
Muscle-dominant wild-type TDP-43 expression induces myopathological changes featuring tubular aggregates and TDP-43-positive inclusions.
Tawara N, Yamashita S, Kawakami K, Kurashige T, Zhang Z, Tasaki M, Yamamoto Y, Nishikami T, Doki T, Zhang X, Matsuo Y, Kimura E, Tawara A, Maeda Y, Hauschka SD, Maruyama H, Ando Y
Experimental neurology 2018 Nov;309:169-180
Experimental neurology 2018 Nov;309:169-180
Age-related deregulation of TDP-43 after stroke enhances NF-κB-mediated inflammation and neuronal damage.
Thammisetty SS, Pedragosa J, Weng YC, Calon F, Planas A, Kriz J
Journal of neuroinflammation 2018 Nov 9;15(1):312
Journal of neuroinflammation 2018 Nov 9;15(1):312
Mutant UBQLN2(P497H) in motor neurons leads to ALS-like phenotypes and defective autophagy in rats.
Chen T, Huang B, Shi X, Gao L, Huang C
Acta neuropathologica communications 2018 Nov 8;6(1):122
Acta neuropathologica communications 2018 Nov 8;6(1):122
Liquid Nuclear Condensates Mechanically Sense and Restructure the Genome.
Shin Y, Chang YC, Lee DSW, Berry J, Sanders DW, Ronceray P, Wingreen NS, Haataja M, Brangwynne CP
Cell 2018 Nov 29;175(6):1481-1491.e13
Cell 2018 Nov 29;175(6):1481-1491.e13
Angiopoietin-1 and ανβ3 integrin peptide promote the therapeutic effects of L-serine in an amyotrophic lateral sclerosis/Parkinsonism dementia complex model.
Cai HY, Tian KW, Zhang YY, Jiang H, Han S
Aging 2018 Nov 25;10(11):3507-3527
Aging 2018 Nov 25;10(11):3507-3527
Active nuclear import and passive nuclear export are the primary determinants of TDP-43 localization.
Pinarbasi ES, Cağatay T, Fung HYJ, Li YC, Chook YM, Thomas PJ
Scientific reports 2018 May 4;8(1):7083
Scientific reports 2018 May 4;8(1):7083
TDP-43 regulation of stress granule dynamics in neurodegenerative disease-relevant cell types.
Khalfallah Y, Kuta R, Grasmuck C, Prat A, Durham HD, Vande Velde C
Scientific reports 2018 May 15;8(1):7551
Scientific reports 2018 May 15;8(1):7551
Essential role of hippocampal noradrenaline in the regulation of spatial working memory and TDP-43 tissue pathology.
Pintus R, Riggi M, Cannarozzo C, Valeri A, de Leo G, Romano M, Gulino R, Leanza G
The Journal of comparative neurology 2018 May 1;526(7):1131-1147
The Journal of comparative neurology 2018 May 1;526(7):1131-1147
TDP-43 regulates the alternative splicing of hnRNP A1 to yield an aggregation-prone variant in amyotrophic lateral sclerosis.
Deshaies JE, Shkreta L, Moszczynski AJ, Sidibé H, Semmler S, Fouillen A, Bennett ER, Bekenstein U, Destroismaisons L, Toutant J, Delmotte Q, Volkening K, Stabile S, Aulas A, Khalfallah Y, Soreq H, Nanci A, Strong MJ, Chabot B, Vande Velde C
Brain : a journal of neurology 2018 May 1;141(5):1320-1333
Brain : a journal of neurology 2018 May 1;141(5):1320-1333
Overlapping Role of SCYL1 and SCYL3 in Maintaining Motor Neuron Viability.
Kuliyev E, Gingras S, Guy CS, Howell S, Vogel P, Pelletier S
The Journal of neuroscience : the official journal of the Society for Neuroscience 2018 Mar 7;38(10):2615-2630
The Journal of neuroscience : the official journal of the Society for Neuroscience 2018 Mar 7;38(10):2615-2630
Lymphoblastoid cell lines as a model to understand amyotrophic lateral sclerosis disease mechanisms.
Pansarasa O, Bordoni M, Drufuca L, Diamanti L, Sproviero D, Trotti R, Bernuzzi S, La Salvia S, Gagliardi S, Ceroni M, Cereda C
Disease models & mechanisms 2018 Mar 26;11(3)
Disease models & mechanisms 2018 Mar 26;11(3)
Murine knockin model for progranulin-deficient frontotemporal dementia with nonsense-mediated mRNA decay.
Nguyen AD, Nguyen TA, Zhang J, Devireddy S, Zhou P, Karydas AM, Xu X, Miller BL, Rigo F, Ferguson SM, Huang EJ, Walther TC, Farese RV Jr
Proceedings of the National Academy of Sciences of the United States of America 2018 Mar 20;115(12):E2849-E2858
Proceedings of the National Academy of Sciences of the United States of America 2018 Mar 20;115(12):E2849-E2858
TDP43 nuclear export and neurodegeneration in models of amyotrophic lateral sclerosis and frontotemporal dementia.
Archbold HC, Jackson KL, Arora A, Weskamp K, Tank EM, Li X, Miguez R, Dayton RD, Tamir S, Klein RL, Barmada SJ
Scientific reports 2018 Mar 15;8(1):4606
Scientific reports 2018 Mar 15;8(1):4606
Zc3h13 Regulates Nuclear RNA m(6)A Methylation and Mouse Embryonic Stem Cell Self-Renewal.
Wen J, Lv R, Ma H, Shen H, He C, Wang J, Jiao F, Liu H, Yang P, Tan L, Lan F, Shi YG, He C, Shi Y, Diao J
Molecular cell 2018 Mar 15;69(6):1028-1038.e6
Molecular cell 2018 Mar 15;69(6):1028-1038.e6
A single N-terminal phosphomimic disrupts TDP-43 polymerization, phase separation, and RNA splicing.
Wang A, Conicella AE, Schmidt HB, Martin EW, Rhoads SN, Reeb AN, Nourse A, Ramirez Montero D, Ryan VH, Rohatgi R, Shewmaker F, Naik MT, Mittag T, Ayala YM, Fawzi NL
The EMBO journal 2018 Mar 1;37(5)
The EMBO journal 2018 Mar 1;37(5)
DCTN1 F52L mutation case of Perry syndrome with progressive supranuclear palsy-like tauopathy.
Honda H, Sasagasako N, Shen C, Shijo M, Hamasaki H, Suzuki SO, Tsuboi Y, Fujii N, Iwaki T
Parkinsonism & related disorders 2018 Jun;51:105-110
Parkinsonism & related disorders 2018 Jun;51:105-110
Frontotemporal dementia with trans-activation response DNA-binding protein 43 presenting with catatonic syndrome.
Watanabe R, Kawakami I, Onaya M, Higashi S, Arai N, Akiyama H, Hasegawa M, Arai T
Neuropathology : official journal of the Japanese Society of Neuropathology 2018 Jun;38(3):281-287
Neuropathology : official journal of the Japanese Society of Neuropathology 2018 Jun;38(3):281-287
Selective Spatiotemporal Vulnerability of Central Nervous System Neurons to Pathologic TAR DNA-Binding Protein 43 in Aged Transgenic Mice.
van Hummel A, Chan G, van der Hoven J, Morsch M, Ippati S, Suh L, Bi M, Asih PR, Lee WS, Butler TA, Przybyla M, Halliday GM, Piguet O, Kiernan MC, Chung RS, Ittner LM, Ke YD
The American journal of pathology 2018 Jun;188(6):1447-1456
The American journal of pathology 2018 Jun;188(6):1447-1456
Accumulation of poly(A) RNA in nuclear granules enriched in Sam68 in motor neurons from the SMNΔ7 mouse model of SMA.
Narcís JO, Tapia O, Tarabal O, Piedrafita L, Calderó J, Berciano MT, Lafarga M
Scientific reports 2018 Jun 25;8(1):9646
Scientific reports 2018 Jun 25;8(1):9646
Mice with endogenous TDP-43 mutations exhibit gain of splicing function and characteristics of amyotrophic lateral sclerosis.
Fratta P, Sivakumar P, Humphrey J, Lo K, Ricketts T, Oliveira H, Brito-Armas JM, Kalmar B, Ule A, Yu Y, Birsa N, Bodo C, Collins T, Conicella AE, Mejia Maza A, Marrero-Gagliardi A, Stewart M, Mianne J, Corrochano S, Emmett W, Codner G, Groves M, Fukumura R, Gondo Y, Lythgoe M, Pauws E, Peskett E, Stanier P, Teboul L, Hallegger M, Calvo A, Chiò A, Isaacs AM, Fawzi NL, Wang E, Housman DE, Baralle F, Greensmith L, Buratti E, Plagnol V, Fisher EM, Acevedo-Arozena A
The EMBO journal 2018 Jun 1;37(11)
The EMBO journal 2018 Jun 1;37(11)
Protective paraspeckle hyper-assembly downstream of TDP-43 loss of function in amyotrophic lateral sclerosis.
Shelkovnikova TA, Kukharsky MS, An H, Dimasi P, Alexeeva S, Shabir O, Heath PR, Buchman VL
Molecular neurodegeneration 2018 Jun 1;13(1):30
Molecular neurodegeneration 2018 Jun 1;13(1):30
Motor neuron differentiation of iPSCs obtained from peripheral blood of a mutant TARDBP ALS patient.
Bossolasco P, Sassone F, Gumina V, Peverelli S, Garzo M, Silani V
Stem cell research 2018 Jul;30:61-68
Stem cell research 2018 Jul;30:61-68
A patient with posterior cortical atrophy due to Alzheimer's disease.
de Souza RKM, Grinberg LT, Josviak ND, de Almeida DB, Ramina R, Kowacs PA, Caramelli P
Dementia & neuropsychologia 2018 Jul-Sep;12(3):326-328
Dementia & neuropsychologia 2018 Jul-Sep;12(3):326-328
The age of onset and evolution of Braak tangle stage and Thal amyloid pathology of Alzheimer's disease in individuals with Down syndrome.
Davidson YS, Robinson A, Prasher VP, Mann DMA
Acta neuropathologica communications 2018 Jul 4;6(1):56
Acta neuropathologica communications 2018 Jul 4;6(1):56
Unexpected similarities between C9ORF72 and sporadic forms of ALS/FTD suggest a common disease mechanism.
Conlon EG, Fagegaltier D, Agius P, Davis-Porada J, Gregory J, Hubbard I, Kang K, Kim D, New York Genome Center ALS Consortium, Phatnani H, Shneider NA, Manley JL
eLife 2018 Jul 13;7
eLife 2018 Jul 13;7
Neuron-Specific HuR-Deficient Mice Spontaneously Develop Motor Neuron Disease.
Sun K, Li X, Chen X, Bai Y, Zhou G, Kokiko-Cochran ON, Lamb B, Hamilton TA, Lin CY, Lee YS, Herjan T
Journal of immunology (Baltimore, Md. : 1950) 2018 Jul 1;201(1):157-166
Journal of immunology (Baltimore, Md. : 1950) 2018 Jul 1;201(1):157-166
Alzheimer neuropathology without frontotemporal lobar degeneration hallmarks (TAR DNA-binding protein 43 inclusions) in missense progranulin mutation Cys139Arg.
Redaelli V, Rossi G, Maderna E, Kovacs GG, Piccoli E, Caroppo P, Cacciatore F, Spinello S, Grisoli M, Sozzi G, Salmaggi A, Tagliavini F, Giaccone G
Brain pathology (Zurich, Switzerland) 2018 Jan;28(1):72-76
Brain pathology (Zurich, Switzerland) 2018 Jan;28(1):72-76
A proteomic network approach across the ALS-FTD disease spectrum resolves clinical phenotypes and genetic vulnerability in human brain.
Umoh ME, Dammer EB, Dai J, Duong DM, Lah JJ, Levey AI, Gearing M, Glass JD, Seyfried NT
EMBO molecular medicine 2018 Jan;10(1):48-62
EMBO molecular medicine 2018 Jan;10(1):48-62
Characterization of Dopaminergic System in the Striatum of Young Adult Park2(-/-) Knockout Rats.
Gemechu JM, Sharma A, Yu D, Xie Y, Merkel OM, Moszczynska A
Scientific reports 2018 Jan 24;8(1):1517
Scientific reports 2018 Jan 24;8(1):1517
Mutation-dependent aggregation and toxicity in a Drosophila model for UBQLN2-associated ALS.
Kim SH, Stiles SG, Feichtmeier JM, Ramesh N, Zhan L, Scalf MA, Smith LM, Pandey UB, Tibbetts RS
Human molecular genetics 2018 Jan 15;27(2):322-337
Human molecular genetics 2018 Jan 15;27(2):322-337
Anterior Cingulate Cortex TDP-43 Pathology in Sporadic Amyotrophic Lateral Sclerosis.
Braak H, Del Tredici K
Journal of neuropathology and experimental neurology 2018 Jan 1;77(1):74-83
Journal of neuropathology and experimental neurology 2018 Jan 1;77(1):74-83
Repetitive Mild Closed Head Injury Alters Protein Expression and Dendritic Complexity in a Mouse Model.
Saykally JN, Ratliff WA, Keeley KL, Pick CG, Mervis RF, Citron BA
Journal of neurotrauma 2018 Jan 1;35(1):139-148
Journal of neurotrauma 2018 Jan 1;35(1):139-148
Protein Phosphatase 1 dephosphorylates TDP-43 and suppresses its function in tau exon 10 inclusion.
Gu J, Wang W, Miao S, Chen F, Wu F, Hu W, Iqbal K, Gong CX, Liu F
FEBS letters 2018 Feb;592(3):402-410
FEBS letters 2018 Feb;592(3):402-410
Angiogenin Prevents Progranulin A9D Mutation-Induced Neuronal-Like Cell Apoptosis Through Cleaving tRNAs into tiRNAs.
Li S, Chen Y, Sun D, Bai R, Gao X, Yang Y, Sheng J, Xu Z
Molecular neurobiology 2018 Feb;55(2):1338-1351
Molecular neurobiology 2018 Feb;55(2):1338-1351
Mutation in the RRM2 domain of TDP-43 in Amyotrophic Lateral Sclerosis with rapid progression associated with ubiquitin positive aggregates in cultured motor neurons.
Maurel C, Madji-Hounoum B, Thepault RA, Marouillat S, Brulard C, Danel-Brunaud V, Camdessanche JP, Blasco H, Corcia P, Andres CR, Vourc'h P
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Feb;19(1-2):149-151
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Feb;19(1-2):149-151
Cerebral ischemia induces the aggregation of proteins linked to neurodegenerative diseases.
Kahl A, Blanco I, Jackman K, Baskar J, Milaganur Mohan H, Rodney-Sandy R, Zhang S, Iadecola C, Hochrainer K
Scientific reports 2018 Feb 9;8(1):2701
Scientific reports 2018 Feb 9;8(1):2701
Citrullination of RGG Motifs in FET Proteins by PAD4 Regulates Protein Aggregation and ALS Susceptibility.
Tanikawa C, Ueda K, Suzuki A, Iida A, Nakamura R, Atsuta N, Tohnai G, Sobue G, Saichi N, Momozawa Y, Kamatani Y, Kubo M, Yamamoto K, Nakamura Y, Matsuda K
Cell reports 2018 Feb 6;22(6):1473-1483
Cell reports 2018 Feb 6;22(6):1473-1483
CHCHD10 mutations p.R15L and p.G66V cause motoneuron disease by haploinsufficiency.
Brockmann SJ, Freischmidt A, Oeckl P, Müller K, Ponna SK, Helferich AM, Paone C, Reinders J, Kojer K, Orth M, Jokela M, Auranen M, Udd B, Hermann A, Danzer KM, Lichtner P, Walther P, Ludolph AC, Andersen PM, Otto M, Kursula P, Just S, Weishaupt JH
Human molecular genetics 2018 Feb 15;27(4):706-715
Human molecular genetics 2018 Feb 15;27(4):706-715
Behavioral defects in a DCTN1(G71A) transgenic mouse model of Perry syndrome.
Mishima T, Deshimaru M, Watanabe T, Kubota K, Kinoshita-Kawada M, Yuasa-Kawada J, Takasaki K, Uehara Y, Jinno S, Iwasaki K, Tsuboi Y
Neuroscience letters 2018 Feb 14;666:98-103
Neuroscience letters 2018 Feb 14;666:98-103
Multiple Proteinopathies in Familial ALS Cases With Optineurin Mutations.
Ayaki T, Ito H, Komure O, Kamada M, Nakamura M, Wate R, Kusaka H, Yamaguchi Y, Li F, Kawakami H, Urushitani M, Takahashi R
Journal of neuropathology and experimental neurology 2018 Feb 1;77(2):128-138
Journal of neuropathology and experimental neurology 2018 Feb 1;77(2):128-138
Mitochondrial abnormalities and disruption of the neuromuscular junction precede the clinical phenotype and motor neuron loss in hFUSWT transgenic mice.
So E, Mitchell JC, Memmi C, Chennell G, Vizcay-Barrena G, Allison L, Shaw CE, Vance C
Human molecular genetics 2018 Feb 1;27(3):463-474
Human molecular genetics 2018 Feb 1;27(3):463-474
TDP-43 pathology in multiple system atrophy: colocalization of TDP-43 and α-synuclein in glial cytoplasmic inclusions.
Koga S, Lin WL, Walton RL, Ross OA, Dickson DW
Neuropathology and applied neurobiology 2018 Dec;44(7):707-721
Neuropathology and applied neurobiology 2018 Dec;44(7):707-721
Enteroviral Infection Leads to Transactive Response DNA-Binding Protein 43 Pathology in Vivo.
Xue YC, Ruller CM, Fung G, Mohamud Y, Deng H, Liu H, Zhang J, Feuer R, Luo H
The American journal of pathology 2018 Dec;188(12):2853-2862
The American journal of pathology 2018 Dec;188(12):2853-2862
Transcription-Dependent Formation of Nuclear Granules Containing FUS and RNA Pol II.
Thompson VF, Victor RA, Morera AA, Moinpour M, Liu MN, Kisiel CC, Pickrel K, Springhower CE, Schwartz JC
Biochemistry 2018 Dec 26;57(51):7021-7032
Biochemistry 2018 Dec 26;57(51):7021-7032
Dichotomous scoring of TDP-43 proteinopathy from specific brain regions in 27 academic research centers: associations with Alzheimer's disease and cerebrovascular disease pathologies.
Katsumata Y, Fardo DW, Kukull WA, Nelson PT
Acta neuropathologica communications 2018 Dec 19;6(1):142
Acta neuropathologica communications 2018 Dec 19;6(1):142
Identification of TCERG1 as a new genetic modulator of TDP-43 production in Drosophila.
Pons M, Prieto S, Miguel L, Frebourg T, Campion D, Suñé C, Lecourtois M
Acta neuropathologica communications 2018 Dec 12;6(1):138
Acta neuropathologica communications 2018 Dec 12;6(1):138
Primary fibroblasts derived from sporadic amyotrophic lateral sclerosis patients do not show ALS cytological lesions.
Codron P, Cassereau J, Vourc'h P, Veyrat-Durebex C, Blasco H, Kane S, Procaccio V, Letournel F, Verny C, Lenears G, Reynier P, Chevrollier A
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Aug;19(5-6):446-456
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Aug;19(5-6):446-456
Analysis of endocannabinoid receptors and enzymes in the post-mortem motor cortex and spinal cord of amyotrophic lateral sclerosis patients.
Espejo-Porras F, Fernández-Ruiz J, de Lago E
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Aug;19(5-6):377-386
Amyotrophic lateral sclerosis & frontotemporal degeneration 2018 Aug;19(5-6):377-386
Disruption by SaCas9 Endonuclease of HERV-Kenv, a Retroviral Gene with Oncogenic and Neuropathogenic Potential, Inhibits Molecules Involved in Cancer and Amyotrophic Lateral Sclerosis.
Ibba G, Piu C, Uleri E, Serra C, Dolei A
Viruses 2018 Aug 7;10(8)
Viruses 2018 Aug 7;10(8)
Transactive Response DNA-Binding Protein 43 Abnormalities after Traumatic Brain Injury.
Tan XL, Sun M, Brady RD, Liu S, Llanos R, Cheung S, Wright DK, Casillas-Espinosa PM, Sashindranath M, O'Brien TJ, McDonald SJ, Turner BJ, Shultz SR
Journal of neurotrauma 2018 Aug 10;
Journal of neurotrauma 2018 Aug 10;
Potentiating Hsp104 activity via phosphomimetic mutations in the middle domain.
Tariq A, Lin J, Noll MM, Torrente MP, Mack KL, Murillo OH, Jackrel ME, Shorter J
FEMS yeast research 2018 Aug 1;18(5)
FEMS yeast research 2018 Aug 1;18(5)
RNA and Protein Interactors with TDP-43 in Human Spinal-Cord Lysates in Amyotrophic Lateral Sclerosis.
Volkening K, Keller BA, Leystra-Lantz C, Strong MJ
Journal of proteome research 2018 Apr 6;17(4):1712-1729
Journal of proteome research 2018 Apr 6;17(4):1712-1729
Neurodegenerative Disease Proteinopathies Are Connected to Distinct Histone Post-translational Modification Landscapes.
Chen K, Bennett SA, Rana N, Yousuf H, Said M, Taaseen S, Mendo N, Meltser SM, Torrente MP
ACS chemical neuroscience 2018 Apr 18;9(4):838-848
ACS chemical neuroscience 2018 Apr 18;9(4):838-848
Phosphorylated TDP-43 (pTDP-43) aggregates in the axial skeletal muscle of patients with sporadic and familial amyotrophic lateral sclerosis.
Cykowski MD, Powell SZ, Appel JW, Arumanayagam AS, Rivera AL, Appel SH
Acta neuropathologica communications 2018 Apr 13;6(1):28
Acta neuropathologica communications 2018 Apr 13;6(1):28
Human Endogenous Retrovirus-K and TDP-43 Expression Bridges ALS and HIV Neuropathology.
Douville RN, Nath A
Frontiers in microbiology 2017;8:1986
Frontiers in microbiology 2017;8:1986
Diabetes is Not Associated with Alzheimer's Disease Neuropathology.
Dos Santos Matioli MNP, Suemoto CK, Rodriguez RD, Farias DS, da Silva MM, Leite REP, Ferretti-Rebustini REL, Farfel JM, Pasqualucci CA, Jacob Filho W, Arvanitakis Z, Naslavsky MS, Zatz M, Grinberg LT, Nitrini R
Journal of Alzheimer's disease : JAD 2017;60(3):1035-1043
Journal of Alzheimer's disease : JAD 2017;60(3):1035-1043
Microglial and Neuronal TDP-43 Pathology in Anti-IgLON5-Related Tauopathy.
Cagnin A, Mariotto S, Fiorini M, Gaule M, Bonetto N, Tagliapietra M, Buratti E, Zanusso G, Ferrari S, Monaco S
Journal of Alzheimer's disease : JAD 2017;59(1):13-20
Journal of Alzheimer's disease : JAD 2017;59(1):13-20
Tau Rather than TDP-43 Proteins are Potential Cerebrospinal Fluid Biomarkers for Frontotemporal Lobar Degeneration Subtypes: A Pilot Study.
Kuiperij HB, Versleijen AA, Beenes M, Verwey NA, Benussi L, Paterlini A, Binetti G, Teunissen CE, Raaphorst J, Schelhaas HJ, Küsters B, Pijnenburg YA, Ghidoni R, Verbeek MM
Journal of Alzheimer's disease : JAD 2017;55(2):585-595
Journal of Alzheimer's disease : JAD 2017;55(2):585-595
Total proteome turbidity assay for tracking global protein aggregation in the natural cellular environment.
Shmueli MD, Hizkiahou N, Peled S, Gazit E, Segal D
Journal of biological methods 2017;4(2):e69
Journal of biological methods 2017;4(2):e69
Regional correlations between [(11)C]PIB PET and post-mortem burden of amyloid-beta pathology in a diverse neuropathological cohort.
Seo SW, Ayakta N, Grinberg LT, Villeneuve S, Lehmann M, Reed B, DeCarli C, Miller BL, Rosen HJ, Boxer AL, O'Neil JP, Jin LW, Seeley WW, Jagust WJ, Rabinovici GD
NeuroImage. Clinical 2017;13:130-137
NeuroImage. Clinical 2017;13:130-137
Truncation of the TAR DNA-binding protein 43 is not a prerequisite for cytoplasmic relocalization, and is suppressed by caspase inhibition and by introduction of the A90V sequence variant.
Wobst HJ, Delsing L, Brandon NJ, Moss SJ
PloS one 2017;12(5):e0177181
PloS one 2017;12(5):e0177181
Neurotrophic effects of progranulin in vivo in reversing motor neuron defects caused by over or under expression of TDP-43 or FUS.
Chitramuthu BP, Kay DG, Bateman A, Bennett HP
PloS one 2017;12(3):e0174784
PloS one 2017;12(3):e0174784
Decreased Levels of Foldase and Chaperone Proteins Are Associated with an Early-Onset Amyotrophic Lateral Sclerosis.
Filareti M, Luotti S, Pasetto L, Pignataro M, Paolella K, Messina P, Pupillo E, Filosto M, Lunetta C, Mandrioli J, Fuda G, Calvo A, Chiò A, Corbo M, Bendotti C, Beghi E, Bonetto V
Frontiers in molecular neuroscience 2017;10:99
Frontiers in molecular neuroscience 2017;10:99
Cytoplasmic Relocalization of TAR DNA-Binding Protein 43 Is Not Sufficient to Reproduce Cellular Pathologies Associated with ALS In vitro.
Wobst HJ, Wesolowski SS, Chadchankar J, Delsing L, Jacobsen S, Mukherjee J, Deeb TZ, Dunlop J, Brandon NJ, Moss SJ
Frontiers in molecular neuroscience 2017;10:46
Frontiers in molecular neuroscience 2017;10:46
Calpain-Dependent Degradation of Nucleoporins Contributes to Motor Neuron Death in a Mouse Model of Chronic Excitotoxicity.
Sugiyama K, Aida T, Nomura M, Takayanagi R, Zeilhofer HU, Tanaka K
The Journal of neuroscience : the official journal of the Society for Neuroscience 2017 Sep 6;37(36):8830-8844
The Journal of neuroscience : the official journal of the Society for Neuroscience 2017 Sep 6;37(36):8830-8844
Amygdala TDP-43 Pathology in Frontotemporal Lobar Degeneration and Motor Neuron Disease.
Takeda T, Seilhean D, Le Ber I, Millecamps S, Sazdovitch V, Kitagawa K, Uchihara T, Duyckaerts C
Journal of neuropathology and experimental neurology 2017 Sep 1;76(9):800-812
Journal of neuropathology and experimental neurology 2017 Sep 1;76(9):800-812
Pathologic Involvement of Glutamatergic Striatal Inputs From the Cortices in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, Ishigaki S, Katsuno M, Sobue G
Journal of neuropathology and experimental neurology 2017 Sep 1;76(9):759-768
Journal of neuropathology and experimental neurology 2017 Sep 1;76(9):759-768
Traumatic Brain Injury Results in Cellular, Structural and Functional Changes Resembling Motor Neuron Disease.
Wright DK, Liu S, van der Poel C, McDonald SJ, Brady RD, Taylor L, Yang L, Gardner AJ, Ordidge R, O'Brien TJ, Johnston LA, Shultz SR
Cerebral cortex (New York, N.Y. : 1991) 2017 Sep 1;27(9):4503-4515
Cerebral cortex (New York, N.Y. : 1991) 2017 Sep 1;27(9):4503-4515
Splicing factors act as genetic modulators of TDP-43 production in a new autoregulatory TDP-43 Drosophila model.
Pons M, Miguel L, Miel C, Avequin T, Juge F, Frebourg T, Campion D, Lecourtois M
Human molecular genetics 2017 Sep 1;26(17):3396-3408
Human molecular genetics 2017 Sep 1;26(17):3396-3408
(18) F-flortaucipir tau positron emission tomography distinguishes established progressive supranuclear palsy from controls and Parkinson disease: A multicenter study.
Schonhaut DR, McMillan CT, Spina S, Dickerson BC, Siderowf A, Devous MD Sr, Tsai R, Winer J, Russell DS, Litvan I, Roberson ED, Seeley WW, Grinberg LT, Kramer JH, Miller BL, Pressman P, Nasrallah I, Baker SL, Gomperts SN, Johnson KA, Grossman M, Jagust WJ, Boxer AL, Rabinovici GD
Annals of neurology 2017 Oct;82(4):622-634
Annals of neurology 2017 Oct;82(4):622-634
The Effect of Different Types of Nanoparticles on FUS and TDP-43 Solubility and Subcellular Localization.
Lojk J, Prpar Mihevc S, Bregar VB, Pavlin M, Rogelj B
Neurotoxicity research 2017 Oct;32(3):325-339
Neurotoxicity research 2017 Oct;32(3):325-339
Association between diabetes and causes of dementia: Evidence from a clinicopathological study.
Matioli MNPDS, Suemoto CK, Rodriguez RD, Farias DS, da Silva MM, Leite REP, Ferretti-Rebustini REL, Pasqualucci CA, Jacob W Filho, Grinberg LT, Nitrini R
Dementia & neuropsychologia 2017 Oct-Dec;11(4):406-412
Dementia & neuropsychologia 2017 Oct-Dec;11(4):406-412
Disulfide cross-linked multimers of TDP-43 and spinal motoneuron loss in a TDP-43(A315T) ALS/FTD mouse model.
Bargsted L, Medinas DB, Martínez Traub F, Rozas P, Muñoz N, Nassif M, Jerez C, Catenaccio A, Court FA, Hetz C, Matus S
Scientific reports 2017 Oct 27;7(1):14266
Scientific reports 2017 Oct 27;7(1):14266
Identification of functional tetramolecular RNA G-quadruplexes derived from transfer RNAs.
Lyons SM, Gudanis D, Coyne SM, Gdaniec Z, Ivanov P
Nature communications 2017 Oct 24;8(1):1127
Nature communications 2017 Oct 24;8(1):1127
Novel miR-b2122 regulates several ALS-related RNA-binding proteins.
Hawley ZCE, Campos-Melo D, Strong MJ
Molecular brain 2017 Oct 2;10(1):46
Molecular brain 2017 Oct 2;10(1):46
Cis P-tau is induced in clinical and preclinical brain injury and contributes to post-injury sequelae.
Albayram O, Kondo A, Mannix R, Smith C, Tsai CY, Li C, Herbert MK, Qiu J, Monuteaux M, Driver J, Yan S, Gormley W, Puccio AM, Okonkwo DO, Lucke-Wold B, Bailes J, Meehan W, Zeidel M, Lu KP, Zhou XZ
Nature communications 2017 Oct 17;8(1):1000
Nature communications 2017 Oct 17;8(1):1000
Nuclear poly(A) binding protein 1 (PABPN1) and Matrin3 interact in muscle cells and regulate RNA processing.
Banerjee A, Vest KE, Pavlath GK, Corbett AH
Nucleic acids research 2017 Oct 13;45(18):10706-10725
Nucleic acids research 2017 Oct 13;45(18):10706-10725
Platelet phosphorylated TDP-43: an exploratory study for a peripheral surrogate biomarker development for Alzheimer's disease.
Wilhite R, Sage JM, Bouzid A, Primavera T, Agbas A
Future science OA 2017 Nov;3(4):FSO238
Future science OA 2017 Nov;3(4):FSO238
Clusterin protects neurons against intracellular proteotoxicity.
Gregory JM, Whiten DR, Brown RA, Barros TP, Kumita JR, Yerbury JJ, Satapathy S, McDade K, Smith C, Luheshi LM, Dobson CM, Wilson MR
Acta neuropathologica communications 2017 Nov 7;5(1):81
Acta neuropathologica communications 2017 Nov 7;5(1):81
TDP-43 accelerates age-dependent degeneration of interneurons.
Tsuiji H, Inoue I, Takeuchi M, Furuya A, Yamakage Y, Watanabe S, Koike M, Hattori M, Yamanaka K
Scientific reports 2017 Nov 2;7(1):14972
Scientific reports 2017 Nov 2;7(1):14972
TDP-43 misexpression causes defects in dendritic growth.
Herzog JJ, Deshpande M, Shapiro L, Rodal AA, Paradis S
Scientific reports 2017 Nov 15;7(1):15656
Scientific reports 2017 Nov 15;7(1):15656
Dysregulation of chromatin remodelling complexes in amyotrophic lateral sclerosis.
Tibshirani M, Zhao B, Gentil BJ, Minotti S, Marques C, Keith J, Rogaeva E, Zinman L, Rouaux C, Robertson J, Durham HD
Human molecular genetics 2017 Nov 1;26(21):4142-4152
Human molecular genetics 2017 Nov 1;26(21):4142-4152
Incipient progressive supranuclear palsy is more common than expected and may comprise clinicopathological subtypes: a forensic autopsy series.
Yoshida K, Hata Y, Kinoshita K, Takashima S, Tanaka K, Nishida N
Acta neuropathologica 2017 May;133(5):809-823
Acta neuropathologica 2017 May;133(5):809-823
Overexpression of the essential Sis1 chaperone reduces TDP-43 effects on toxicity and proteolysis.
Park SK, Hong JY, Arslan F, Kanneganti V, Patel B, Tietsort A, Tank EMH, Li X, Barmada SJ, Liebman SW
PLoS genetics 2017 May;13(5):e1006805
PLoS genetics 2017 May;13(5):e1006805
Dioxins and related environmental contaminants increase TDP-43 levels.
Ash PEA, Stanford EA, Al Abdulatif A, Ramirez-Cardenas A, Ballance HI, Boudeau S, Jeh A, Murithi JM, Tripodis Y, Murphy GJ, Sherr DH, Wolozin B
Molecular neurodegeneration 2017 May 5;12(1):35
Molecular neurodegeneration 2017 May 5;12(1):35
Progressive Motor Neuron Pathology and the Role of Astrocytes in a Human Stem Cell Model of VCP-Related ALS.
Hall CE, Yao Z, Choi M, Tyzack GE, Serio A, Luisier R, Harley J, Preza E, Arber C, Crisp SJ, Watson PMD, Kullmann DM, Abramov AY, Wray S, Burley R, Loh SHY, Martins LM, Stevens MM, Luscombe NM, Sibley CR, Lakatos A, Ule J, Gandhi S, Patani R
Cell reports 2017 May 30;19(9):1739-1749
Cell reports 2017 May 30;19(9):1739-1749
Clinical Significance of TDP-43 Neuropathology in Amyotrophic Lateral Sclerosis.
Cykowski MD, Powell SZ, Peterson LE, Appel JW, Rivera AL, Takei H, Chang E, Appel SH
Journal of neuropathology and experimental neurology 2017 May 1;76(5):402-413
Journal of neuropathology and experimental neurology 2017 May 1;76(5):402-413
Absence of Alzheimer Disease Neuropathologic Changes in Eyes of Subjects With Alzheimer Disease.
Williams EA, McGuone D, Frosch MP, Hyman BT, Laver N, Stemmer-Rachamimov A
Journal of neuropathology and experimental neurology 2017 May 1;76(5):376-383
Journal of neuropathology and experimental neurology 2017 May 1;76(5):376-383
TDP-43 mutations causing amyotrophic lateral sclerosis are associated with altered expression of RNA-binding protein hnRNP K and affect the Nrf2 antioxidant pathway.
Moujalled D, Grubman A, Acevedo K, Yang S, Ke YD, Moujalled DM, Duncan C, Caragounis A, Perera ND, Turner BJ, Prudencio M, Petrucelli L, Blair I, Ittner LM, Crouch PJ, Liddell JR, White AR
Human molecular genetics 2017 May 1;26(9):1732-1746
Human molecular genetics 2017 May 1;26(9):1732-1746
Loss of Ranbp2 in motoneurons causes disruption of nucleocytoplasmic and chemokine signaling, proteostasis of hnRNPH3 and Mmp28, and development of amyotrophic lateral sclerosis-like syndromes.
Cho KI, Yoon D, Qiu S, Danziger Z, Grill WM, Wetsel WC, Ferreira PA
Disease models & mechanisms 2017 May 1;10(5):559-579
Disease models & mechanisms 2017 May 1;10(5):559-579
Neuropathological diagnoses and clinical correlates in older adults in Brazil: A cross-sectional study.
Suemoto CK, Ferretti-Rebustini RE, Rodriguez RD, Leite RE, Soterio L, Brucki SM, Spera RR, Cippiciani TM, Farfel JM, Chiavegatto Filho A, Naslavsky MS, Zatz M, Pasqualucci CA, Jacob-Filho W, Nitrini R, Grinberg LT
PLoS medicine 2017 Mar;14(3):e1002267
PLoS medicine 2017 Mar;14(3):e1002267
DUX4-induced dsRNA and MYC mRNA stabilization activate apoptotic pathways in human cell models of facioscapulohumeral dystrophy.
Shadle SC, Zhong JW, Campbell AE, Conerly ML, Jagannathan S, Wong CJ, Morello TD, van der Maarel SM, Tapscott SJ
PLoS genetics 2017 Mar;13(3):e1006658
PLoS genetics 2017 Mar;13(3):e1006658
Retrotransposon activation contributes to neurodegeneration in a Drosophila TDP-43 model of ALS.
Krug L, Chatterjee N, Borges-Monroy R, Hearn S, Liao WW, Morrill K, Prazak L, Rozhkov N, Theodorou D, Hammell M, Dubnau J
PLoS genetics 2017 Mar;13(3):e1006635
PLoS genetics 2017 Mar;13(3):e1006635
Single cell imaging and quantification of TDP-43 and α-synuclein intercellular propagation.
Peled S, Sade D, Bram Y, Porat Z, Kreiser T, Mimouni M, Lichtenstein A, Segal D, Gazit E
Scientific reports 2017 Mar 28;7(1):544
Scientific reports 2017 Mar 28;7(1):544
Heat Shock-induced Phosphorylation of TAR DNA-binding Protein 43 (TDP-43) by MAPK/ERK Kinase Regulates TDP-43 Function.
Li W, Reeb AN, Lin B, Subramanian P, Fey EE, Knoverek CR, French RL, Bigio EH, Ayala YM
The Journal of biological chemistry 2017 Mar 24;292(12):5089-5100
The Journal of biological chemistry 2017 Mar 24;292(12):5089-5100
Valosin-containing protein (VCP/p97) inhibitors relieve Mitofusin-dependent mitochondrial defects due to VCP disease mutants.
Zhang T, Mishra P, Hay BA, Chan D, Guo M
eLife 2017 Mar 21;6
eLife 2017 Mar 21;6
Amyotrophic lateral sclerosis-linked mutations increase the viscosity of liquid-like TDP-43 RNP granules in neurons.
Gopal PP, Nirschl JJ, Klinman E, Holzbaur EL
Proceedings of the National Academy of Sciences of the United States of America 2017 Mar 21;114(12):E2466-E2475
Proceedings of the National Academy of Sciences of the United States of America 2017 Mar 21;114(12):E2466-E2475
Simple Derivation of Spinal Motor Neurons from ESCs/iPSCs Using Sendai Virus Vectors.
Goto K, Imamura K, Komatsu K, Mitani K, Aiba K, Nakatsuji N, Inoue M, Kawata A, Yamashita H, Takahashi R, Inoue H
Molecular therapy. Methods & clinical development 2017 Mar 17;4:115-125
Molecular therapy. Methods & clinical development 2017 Mar 17;4:115-125
C9ORF72 hexanucleotide repeat exerts toxicity in a stable, inducible motor neuronal cell model, which is rescued by partial depletion of Pten.
Stopford MJ, Higginbottom A, Hautbergue GM, Cooper-Knock J, Mulcahy PJ, De Vos KJ, Renton AE, Pliner H, Calvo A, Chio A, Traynor BJ, Azzouz M, Heath PR, ITALSGEN Consortium, NeuroX Consortium, Kirby J, Shaw PJ
Human molecular genetics 2017 Mar 15;26(6):1133-1145
Human molecular genetics 2017 Mar 15;26(6):1133-1145
Enhancing Mitofusin/Marf ameliorates neuromuscular dysfunction in Drosophila models of TDP-43 proteinopathies.
Khalil B, Cabirol-Pol MJ, Miguel L, Whitworth AJ, Lecourtois M, Liévens JC
Neurobiology of aging 2017 Jun;54:71-83
Neurobiology of aging 2017 Jun;54:71-83
Cryptic exon incorporation occurs in Alzheimer's brain lacking TDP-43 inclusion but exhibiting nuclear clearance of TDP-43.
Sun M, Bell W, LaClair KD, Ling JP, Han H, Kageyama Y, Pletnikova O, Troncoso JC, Wong PC, Chen LL
Acta neuropathologica 2017 Jun;133(6):923-931
Acta neuropathologica 2017 Jun;133(6):923-931
Heterogeneous ribonuclear protein E2 (hnRNP E2) is associated with TDP-43-immunoreactive neurites in Semantic Dementia but not with other TDP-43 pathological subtypes of Frontotemporal Lobar Degeneration.
Davidson YS, Robinson AC, Flood L, Rollinson S, Benson BC, Asi YT, Richardson A, Jones M, Snowden JS, Pickering-Brown S, Lashley T, Mann DMA
Acta neuropathologica communications 2017 Jun 30;5(1):54
Acta neuropathologica communications 2017 Jun 30;5(1):54
Functional and dynamic polymerization of the ALS-linked protein TDP-43 antagonizes its pathologic aggregation.
Afroz T, Hock EM, Ernst P, Foglieni C, Jambeau M, Gilhespy LAB, Laferriere F, Maniecka Z, Plückthun A, Mittl P, Paganetti P, Allain FHT, Polymenidou M
Nature communications 2017 Jun 29;8(1):45
Nature communications 2017 Jun 29;8(1):45
Dcp1a and GW182 Induce Distinct Cellular Aggregates and Have Different Effects on microRNA Pathway.
Wang X, Chang L, Wang H, Su A, Wu Z
DNA and cell biology 2017 Jul;36(7):565-570
DNA and cell biology 2017 Jul;36(7):565-570
Misfolded SOD1 is not a primary component of sporadic ALS.
Da Cruz S, Bui A, Saberi S, Lee SK, Stauffer J, McAlonis-Downes M, Schulte D, Pizzo DP, Parone PA, Cleveland DW, Ravits J
Acta neuropathologica 2017 Jul;134(1):97-111
Acta neuropathologica 2017 Jul;134(1):97-111
Expansion of the classification of FTLD-TDP: distinct pathology associated with rapidly progressive frontotemporal degeneration.
Lee EB, Porta S, Michael Baer G, Xu Y, Suh E, Kwong LK, Elman L, Grossman M, Lee VM, Irwin DJ, Van Deerlin VM, Trojanowski JQ
Acta neuropathologica 2017 Jul;134(1):65-78
Acta neuropathologica 2017 Jul;134(1):65-78
TDP-43 expression influences amyloidβ plaque deposition and tau aggregation.
Davis SA, Gan KA, Dowell JA, Cairns NJ, Gitcho MA
Neurobiology of disease 2017 Jul;103:154-162
Neurobiology of disease 2017 Jul;103:154-162
Anti-AMPA GluA3 antibodies in Frontotemporal dementia: a new molecular target.
Borroni B, Stanic J, Verpelli C, Mellone M, Bonomi E, Alberici A, Bernasconi P, Culotta L, Zianni E, Archetti S, Manes M, Gazzina S, Ghidoni R, Benussi L, Stuani C, Di Luca M, Sala C, Buratti E, Padovani A, Gardoni F
Scientific reports 2017 Jul 27;7(1):6723
Scientific reports 2017 Jul 27;7(1):6723
Major hnRNP proteins act as general TDP-43 functional modifiers both in Drosophila and human neuronal cells.
Appocher C, Mohagheghi F, Cappelli S, Stuani C, Romano M, Feiguin F, Buratti E
Nucleic acids research 2017 Jul 27;45(13):8026-8045
Nucleic acids research 2017 Jul 27;45(13):8026-8045
TDP-43 Depletion in Microglia Promotes Amyloid Clearance but Also Induces Synapse Loss.
Paolicelli RC, Jawaid A, Henstridge CM, Valeri A, Merlini M, Robinson JL, Lee EB, Rose J, Appel S, Lee VM, Trojanowski JQ, Spires-Jones T, Schulz PE, Rajendran L
Neuron 2017 Jul 19;95(2):297-308.e6
Neuron 2017 Jul 19;95(2):297-308.e6
Acetylation-induced TDP-43 pathology is suppressed by an HSF1-dependent chaperone program.
Wang P, Wander CM, Yuan CX, Bereman MS, Cohen TJ
Nature communications 2017 Jul 19;8(1):82
Nature communications 2017 Jul 19;8(1):82
Point mutations in the N-terminal domain of transactive response DNA-binding protein 43 kDa (TDP-43) compromise its stability, dimerization, and functions.
Mompeán M, Romano V, Pantoja-Uceda D, Stuani C, Baralle FE, Buratti E, Laurents DV
The Journal of biological chemistry 2017 Jul 14;292(28):11992-12006
The Journal of biological chemistry 2017 Jul 14;292(28):11992-12006
Pathological correlations of [F-18]-AV-1451 imaging in non-alzheimer tauopathies.
Marquié M, Normandin MD, Meltzer AC, Siao Tick Chong M, Andrea NV, Antón-Fernández A, Klunk WE, Mathis CA, Ikonomovic MD, Debnath M, Bien EA, Vanderburg CR, Costantino I, Makaretz S, DeVos SL, Oakley DH, Gomperts SN, Growdon JH, Domoto-Reilly K, Lucente D, Dickerson BC, Frosch MP, Hyman BT, Johnson KA, Gómez-Isla T
Annals of neurology 2017 Jan;81(1):117-128
Annals of neurology 2017 Jan;81(1):117-128
Pathological TDP-43 changes in Betz cells differ from those in bulbar and spinal α-motoneurons in sporadic amyotrophic lateral sclerosis.
Braak H, Ludolph AC, Neumann M, Ravits J, Del Tredici K
Acta neuropathologica 2017 Jan;133(1):79-90
Acta neuropathologica 2017 Jan;133(1):79-90
Motor-Coordinative and Cognitive Dysfunction Caused by Mutant TDP-43 Could Be Reversed by Inhibiting Its Mitochondrial Localization.
Wang W, Arakawa H, Wang L, Okolo O, Siedlak SL, Jiang Y, Gao J, Xie F, Petersen RB, Wang X
Molecular therapy : the journal of the American Society of Gene Therapy 2017 Jan 4;25(1):127-139
Molecular therapy : the journal of the American Society of Gene Therapy 2017 Jan 4;25(1):127-139
A Multi-network Approach Identifies Protein-Specific Co-expression in Asymptomatic and Symptomatic Alzheimer's Disease.
Seyfried NT, Dammer EB, Swarup V, Nandakumar D, Duong DM, Yin L, Deng Q, Nguyen T, Hales CM, Wingo T, Glass J, Gearing M, Thambisetty M, Troncoso JC, Geschwind DH, Lah JJ, Levey AI
Cell systems 2017 Jan 25;4(1):60-72.e4
Cell systems 2017 Jan 25;4(1):60-72.e4
TDP-43 protein variants as biomarkers in amyotrophic lateral sclerosis.
Williams SM, Khan G, Harris BT, Ravits J, Sierks MR
BMC neuroscience 2017 Jan 25;18(1):20
BMC neuroscience 2017 Jan 25;18(1):20
Imaging specific newly synthesized proteins within cells by fluorescence resonance energy transfer.
Sheng L, Cai L, Liu J, Zhang S, Xu JJ, Zhang X, Chen HY
Chemical science 2017 Jan 1;8(1):748-754
Chemical science 2017 Jan 1;8(1):748-754
Impaired activation of ALS monocytes by exosomes.
Zondler L, Feiler MS, Freischmidt A, Ruf WP, Ludolph AC, Danzer KM, Weishaupt JH
Immunology and cell biology 2017 Feb;95(2):207-214
Immunology and cell biology 2017 Feb;95(2):207-214
Tdp-43 cryptic exons are highly variable between cell types.
Jeong YH, Ling JP, Lin SZ, Donde AN, Braunstein KE, Majounie E, Traynor BJ, LaClair KD, Lloyd TE, Wong PC
Molecular neurodegeneration 2017 Feb 2;12(1):13
Molecular neurodegeneration 2017 Feb 2;12(1):13
An intronic variation in SLC52A1 causes exon skipping and transient riboflavin-responsive multiple acyl-CoA dehydrogenation deficiency.
Mosegaard S, Bruun GH, Flyvbjerg KF, Bliksrud YT, Gregersen N, Dembic M, Annexstad E, Tangeraas T, Olsen RKJ, Andresen BS
Molecular genetics and metabolism 2017 Dec;122(4):182-188
Molecular genetics and metabolism 2017 Dec;122(4):182-188
Imbalanced expression of polycistronic miRNA in acute myeloid leukemia.
Kotaki R, Higuchi H, Ogiya D, Katahira Y, Kurosaki N, Yukihira N, Ogata J, Yamamoto H, Mohamad Alba S, Azhim A, Kitajima T, Inoue S, Morishita K, Ono K, Koyama-Nasu R, Kotani A
International journal of hematology 2017 Dec;106(6):811-819
International journal of hematology 2017 Dec;106(6):811-819
Clinical and neuropathological features of ALS/FTD with TIA1 mutations.
Hirsch-Reinshagen V, Pottier C, Nicholson AM, Baker M, Hsiung GR, Krieger C, Sengdy P, Boylan KB, Dickson DW, Mesulam M, Weintraub S, Bigio E, Zinman L, Keith J, Rogaeva E, Zivkovic SA, Lacomis D, Taylor JP, Rademakers R, Mackenzie IRA
Acta neuropathologica communications 2017 Dec 7;5(1):96
Acta neuropathologica communications 2017 Dec 7;5(1):96
De novo design of RNA-binding proteins with a prion-like domain related to ALS/FTD proteinopathies.
Mitsuhashi K, Ito D, Mashima K, Oyama M, Takahashi S, Suzuki N
Scientific reports 2017 Dec 4;7(1):16871
Scientific reports 2017 Dec 4;7(1):16871
TDP-43 Promotes Neurodegeneration by Impairing Chromatin Remodeling.
Berson A, Sartoris A, Nativio R, Van Deerlin V, Toledo JB, Porta S, Liu S, Chung CY, Garcia BA, Lee VM, Trojanowski JQ, Johnson FB, Berger SL, Bonini NM
Current biology : CB 2017 Dec 4;27(23):3579-3590.e6
Current biology : CB 2017 Dec 4;27(23):3579-3590.e6
TDP-43 proteolysis is associated with astrocyte reactivity after traumatic brain injury in rodents.
Huang CY, Lee YC, Li PC, Liliang PC, Lu K, Wang KW, Chang LC, Shiu LY, Chen MF, Sun YT, Wang HK
Journal of neuroimmunology 2017 Dec 15;313:61-68
Journal of neuroimmunology 2017 Dec 15;313:61-68
Alcohol-abuse drug disulfiram targets cancer via p97 segregase adaptor NPL4.
Skrott Z, Mistrik M, Andersen KK, Friis S, Majera D, Gursky J, Ozdian T, Bartkova J, Turi Z, Moudry P, Kraus M, Michalova M, Vaclavkova J, Dzubak P, Vrobel I, Pouckova P, Sedlacek J, Miklovicova A, Kutt A, Li J, Mattova J, Driessen C, Dou QP, Olsen J, Hajduch M, Cvek B, Deshaies RJ, Bartek J
Nature 2017 Dec 14;552(7684):194-199
Nature 2017 Dec 14;552(7684):194-199
Endocytosis regulates TDP-43 toxicity and turnover.
Liu G, Coyne AN, Pei F, Vaughan S, Chaung M, Zarnescu DC, Buchan JR
Nature communications 2017 Dec 12;8(1):2092
Nature communications 2017 Dec 12;8(1):2092
GPNMB ameliorates mutant TDP-43-induced motor neuron cell death.
Nagahara Y, Shimazawa M, Ohuchi K, Ito J, Takahashi H, Tsuruma K, Kakita A, Hara H
Journal of neuroscience research 2017 Aug;95(8):1647-1665
Journal of neuroscience research 2017 Aug;95(8):1647-1665
Modelling FUS Mislocalisation in an In Vitro Model of Innervated Human Muscle.
Prpar Mihevc S, Pavlin M, Darovic S, Živin M, Podbregar M, Rogelj B, Mars T
Journal of molecular neuroscience : MN 2017 Aug;62(3-4):318-328
Journal of molecular neuroscience : MN 2017 Aug;62(3-4):318-328
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics.
Mackenzie IR, Nicholson AM, Sarkar M, Messing J, Purice MD, Pottier C, Annu K, Baker M, Perkerson RB, Kurti A, Matchett BJ, Mittag T, Temirov J, Hsiung GR, Krieger C, Murray ME, Kato M, Fryer JD, Petrucelli L, Zinman L, Weintraub S, Mesulam M, Keith J, Zivkovic SA, Hirsch-Reinshagen V, Roos RP, Züchner S, Graff-Radford NR, Petersen RC, Caselli RJ, Wszolek ZK, Finger E, Lippa C, Lacomis D, Stewart H, Dickson DW, Kim HJ, Rogaeva E, Bigio E, Boylan KB, Taylor JP, Rademakers R
Neuron 2017 Aug 16;95(4):808-816.e9
Neuron 2017 Aug 16;95(4):808-816.e9
Perry Syndrome: A Distinctive Type of TDP-43 Proteinopathy.
Mishima T, Koga S, Lin WL, Kasanuki K, Castanedes-Casey M, Wszolek ZK, Oh SJ, Tsuboi Y, Dickson DW
Journal of neuropathology and experimental neurology 2017 Aug 1;76(8):676-682
Journal of neuropathology and experimental neurology 2017 Aug 1;76(8):676-682
VEGF alleviates ALS-CSF induced cytoplasmic accumulations of TDP-43 and FUS/TLS in NSC-34 cells.
Shantanu S, Vijayalakshmi K, Shruthi S, Sagar BKC, Sathyaprabha TN, Nalini A, Raju TR, Alladi PA
Journal of chemical neuroanatomy 2017 Apr;81:48-52
Journal of chemical neuroanatomy 2017 Apr;81:48-52
Regulatory Role of RNA Chaperone TDP-43 for RNA Misfolding and Repeat-Associated Translation in SCA31.
Ishiguro T, Sato N, Ueyama M, Fujikake N, Sellier C, Kanegami A, Tokuda E, Zamiri B, Gall-Duncan T, Mirceta M, Furukawa Y, Yokota T, Wada K, Taylor JP, Pearson CE, Charlet-Berguerand N, Mizusawa H, Nagai Y, Ishikawa K
Neuron 2017 Apr 5;94(1):108-124.e7
Neuron 2017 Apr 5;94(1):108-124.e7
Heterogeneous ribonuclear protein A3 (hnRNP A3) is present in dipeptide repeat protein containing inclusions in Frontotemporal Lobar Degeneration and Motor Neurone disease associated with expansions in C9orf72 gene.
Davidson YS, Flood L, Robinson AC, Nihei Y, Mori K, Rollinson S, Richardson A, Benson BC, Jones M, Snowden JS, Pickering-Brown S, Haass C, Lashley T, Mann DMA
Acta neuropathologica communications 2017 Apr 21;5(1):31
Acta neuropathologica communications 2017 Apr 21;5(1):31
Multireceptor fingerprints in progressive supranuclear palsy.
Chiu WZ, Donker Kaat L, Boon AJW, Kamphorst W, Schleicher A, Zilles K, van Swieten JC, Palomero-Gallagher N
Alzheimer's research & therapy 2017 Apr 17;9(1):28
Alzheimer's research & therapy 2017 Apr 17;9(1):28
Increased cytoplasmic TDP-43 reduces global protein synthesis by interacting with RACK1 on polyribosomes.
Russo A, Scardigli R, La Regina F, Murray ME, Romano N, Dickson DW, Wolozin B, Cattaneo A, Ceci M
Human molecular genetics 2017 Apr 15;26(8):1407-1418
Human molecular genetics 2017 Apr 15;26(8):1407-1418
Survival in the pre-senile dementia frontotemporal lobar degeneration with TDP-43 proteinopathy: effects of genetic, demographic and neuropathological variables.
Armstrong RA
Folia neuropathologica 2016;54(2):137-48
Folia neuropathologica 2016;54(2):137-48
Progression of tau pathology within cholinergic nucleus basalis neurons in chronic traumatic encephalopathy: A chronic effects of neurotrauma consortium study.
Mufson EJ, Perez SE, Nadeem M, Mahady L, Kanaan NM, Abrahamson EE, Ikonomovic MD, Crawford F, Alvarez V, Stein T, McKee AC
Brain injury 2016;30(12):1399-1413
Brain injury 2016;30(12):1399-1413
Mixed tau and TDP-43 pathology in a patient with unclassifiable primary progressive aphasia.
Flanagan EP, Duffy JR, Whitwell JL, Vemuri P, Dickson DW, Josephs KA
Neurocase 2016;22(1):55-9
Neurocase 2016;22(1):55-9
DYT6 Dystonia: A Neuropathological Study.
Paudel R, Li A, Hardy J, Bhatia KP, Houlden H, Holton J
Neuro-degenerative diseases 2016;16(3-4):273-8
Neuro-degenerative diseases 2016;16(3-4):273-8
Shared Molecular Mechanisms in Alzheimer's Disease and Amyotrophic Lateral Sclerosis: Neurofilament-Dependent Transport of sAPP, FUS, TDP-43 and SOD1, with Endoplasmic Reticulum-Like Tubules.
Muresan V, Ladescu Muresan Z
Neuro-degenerative diseases 2016;16(1-2):55-61
Neuro-degenerative diseases 2016;16(1-2):55-61
Detection of hyperphosphorylated tau protein and α-synuclein in spinal cord of patients with Alzheimer's disease.
Guo Y, Wang L, Zhu M, Zhang H, Hu Y, Han Z, Liu J, Zhao W, Wang D
Neuropsychiatric disease and treatment 2016;12:445-52
Neuropsychiatric disease and treatment 2016;12:445-52
Distinct Muscle Biopsy Findings in Genetically Defined Adult-Onset Motor Neuron Disorders.
Jokela M, Huovinen S, Raheem O, Lindfors M, Palmio J, Penttilä S, Udd B
PloS one 2016;11(3):e0151376
PloS one 2016;11(3):e0151376
Cytoplasmic aggregates of dynactin in iPSC-derived tyrosine hydroxylase-positive neurons from a patient with Perry syndrome.
Mishima T, Ishikawa T, Imamura K, Kondo T, Koshiba Y, Takahashi R, Takahashi J, Watanabe A, Fujii N, Tsuboi Y, Inoue H
Parkinsonism & related disorders 2016 Sep;30:67-72
Parkinsonism & related disorders 2016 Sep;30:67-72
Reduced hnRNPA3 increases C9orf72 repeat RNA levels and dipeptide-repeat protein deposition.
Mori K, Nihei Y, Arzberger T, Zhou Q, Mackenzie IR, Hermann A, Hanisch F, German Consortium for Frontotemporal Lobar Degeneration, Bavarian Brain Banking Alliance, Kamp F, Nuscher B, Orozco D, Edbauer D, Haass C
EMBO reports 2016 Sep;17(9):1314-25
EMBO reports 2016 Sep;17(9):1314-25
TDP-43 aggregation mirrors TDP-43 knockdown, affecting the expression levels of a common set of proteins.
Prpar Mihevc S, Baralle M, Buratti E, Rogelj B
Scientific reports 2016 Sep 26;6:33996
Scientific reports 2016 Sep 26;6:33996
Structural, super-resolution microscopy analysis of paraspeckle nuclear body organization.
West JA, Mito M, Kurosaka S, Takumi T, Tanegashima C, Chujo T, Yanaka K, Kingston RE, Hirose T, Bond C, Fox A, Nakagawa S
The Journal of cell biology 2016 Sep 26;214(7):817-30
The Journal of cell biology 2016 Sep 26;214(7):817-30
Enhancing survival motor neuron expression extends lifespan and attenuates neurodegeneration in mutant TDP-43 mice.
Perera ND, Sheean RK, Crouch PJ, White AR, Horne MK, Turner BJ
Human molecular genetics 2016 Sep 15;25(18):4080-4093
Human molecular genetics 2016 Sep 15;25(18):4080-4093
Immunoprecipitation and mass spectrometry defines an extensive RBM45 protein-protein interaction network.
Li Y, Collins M, An J, Geiser R, Tegeler T, Tsantilas K, Garcia K, Pirrotte P, Bowser R
Brain research 2016 Sep 15;1647:79-93
Brain research 2016 Sep 15;1647:79-93
The extreme N-terminus of TDP-43 mediates the cytoplasmic aggregation of TDP-43 and associated toxicity in vivo.
Sasaguri H, Chew J, Xu YF, Gendron TF, Garrett A, Lee CW, Jansen-West K, Bauer PO, Perkerson EA, Tong J, Stetler C, Zhang YJ
Brain research 2016 Sep 15;1647:57-64
Brain research 2016 Sep 15;1647:57-64
Level of PICALM, a key component of clathrin-mediated endocytosis, is correlated with levels of phosphotau and autophagy-related proteins and is associated with tau inclusions in AD, PSP and Pick disease.
Ando K, Tomimura K, Sazdovitch V, Suain V, Yilmaz Z, Authelet M, Ndjim M, Vergara C, Belkouch M, Potier MC, Duyckaerts C, Brion JP
Neurobiology of disease 2016 Oct;94:32-43
Neurobiology of disease 2016 Oct;94:32-43
Axonal TDP-43 aggregates in sporadic amyotrophic lateral sclerosis.
Onozato T, Nakahara A, Suzuki-Kouyama E, Hineno A, Yasude T, Nakamura T, Yahikozawa H, Watanabe M, Kayanuma K, Makishita H, Ohara S, Hashimoto T, Higuchi K, Sakai T, Asano K, Hashimoto T, Kanno H, Nakayama J, Oyanagi K
Neuropathology and applied neurobiology 2016 Oct;42(6):561-72
Neuropathology and applied neurobiology 2016 Oct;42(6):561-72
GRN and MAPT Mutations in 2 Frontotemporal Dementia Research Centers in Brazil.
Takada LT, Bahia VS, Guimarães HC, Costa TV, Vale TC, Rodriguez RD, Porto FH, Machado JC, Beato RG, Cesar KG, Smid J, Nascimento CF, Grinberg LT, Brucki SM, Maximino JR, Camargos ST, Chadi G, Caramelli P, Nitrini R
Alzheimer disease and associated disorders 2016 Oct-Dec;30(4):310-317
Alzheimer disease and associated disorders 2016 Oct-Dec;30(4):310-317
C9orf72 Dipeptide Repeats Impair the Assembly, Dynamics, and Function of Membrane-Less Organelles.
Lee KH, Zhang P, Kim HJ, Mitrea DM, Sarkar M, Freibaum BD, Cika J, Coughlin M, Messing J, Molliex A, Maxwell BA, Kim NC, Temirov J, Moore J, Kolaitis RM, Shaw TI, Bai B, Peng J, Kriwacki RW, Taylor JP
Cell 2016 Oct 20;167(3):774-788.e17
Cell 2016 Oct 20;167(3):774-788.e17
Reduced stress granule formation and cell death in fibroblasts with the A382T mutation of TARDBP gene: evidence for loss of TDP-43 nuclear function.
Orrù S, Coni P, Floris A, Littera R, Carcassi C, Sogos V, Brancia C
Human molecular genetics 2016 Oct 15;25(20):4473-4483
Human molecular genetics 2016 Oct 15;25(20):4473-4483
Role of BMP receptor traffic in synaptic growth defects in an ALS model.
Deshpande M, Feiger Z, Shilton AK, Luo CC, Silverman E, Rodal AA
Molecular biology of the cell 2016 Oct 1;27(19):2898-910
Molecular biology of the cell 2016 Oct 1;27(19):2898-910
Tyrosine kinase inhibition reverses TDP-43 effects on synaptic protein expression, astrocytic function and amino acid dis-homeostasis.
Heyburn L, Hebron ML, Smith J, Winston C, Bechara J, Li Z, Lonskaya I, Burns MP, Harris BT, Moussa CE
Journal of neurochemistry 2016 Nov;139(4):610-623
Journal of neurochemistry 2016 Nov;139(4):610-623
Familial Amyotrophic Lateral Sclerosis-linked Mutations in Profilin 1 Exacerbate TDP-43-induced Degeneration in the Retina of Drosophila melanogaster through an Increase in the Cytoplasmic Localization of TDP-43.
Matsukawa K, Hashimoto T, Matsumoto T, Ihara R, Chihara T, Miura M, Wakabayashi T, Iwatsubo T
The Journal of biological chemistry 2016 Nov 4;291(45):23464-23476
The Journal of biological chemistry 2016 Nov 4;291(45):23464-23476
Unusual Processing Generates SPA LncRNAs that Sequester Multiple RNA Binding Proteins.
Wu H, Yin QF, Luo Z, Yao RW, Zheng CC, Zhang J, Xiang JF, Yang L, Chen LL
Molecular cell 2016 Nov 3;64(3):534-548
Molecular cell 2016 Nov 3;64(3):534-548
Hippocampal Sclerosis but Not Normal Aging or Alzheimer Disease Is Associated With TDP-43 Pathology in the Basal Forebrain of Aged Persons.
Cykowski MD, Takei H, Van Eldik LJ, Schmitt FA, Jicha GA, Powell SZ, Nelson PT
Journal of neuropathology and experimental neurology 2016 May;75(5):397-407
Journal of neuropathology and experimental neurology 2016 May;75(5):397-407
The heat shock response plays an important role in TDP-43 clearance: evidence for dysfunction in amyotrophic lateral sclerosis.
Chen HJ, Mitchell JC, Novoselov S, Miller J, Nishimura AL, Scotter EL, Vance CA, Cheetham ME, Shaw CE
Brain : a journal of neurology 2016 May;139(Pt 5):1417-32
Brain : a journal of neurology 2016 May;139(Pt 5):1417-32
Analysis of RNA metabolism in peripheral WBCs of TDP-43 KI mice identifies novel biomarkers of ALS.
Hasegawa M, Hara-Miyauchi C, Ohta H, Sakimura K, Okano H, Okano HJ
Neuroscience research 2016 May;106:12-22
Neuroscience research 2016 May;106:12-22
Gene-specific mitochondria dysfunctions in human TARDBP and C9ORF72 fibroblasts.
Onesto E, Colombrita C, Gumina V, Borghi MO, Dusi S, Doretti A, Fagiolari G, Invernizzi F, Moggio M, Tiranti V, Silani V, Ratti A
Acta neuropathologica communications 2016 May 5;4(1):47
Acta neuropathologica communications 2016 May 5;4(1):47
Gain of Toxicity from ALS/FTD-Linked Repeat Expansions in C9ORF72 Is Alleviated by Antisense Oligonucleotides Targeting GGGGCC-Containing RNAs.
Jiang J, Zhu Q, Gendron TF, Saberi S, McAlonis-Downes M, Seelman A, Stauffer JE, Jafar-Nejad P, Drenner K, Schulte D, Chun S, Sun S, Ling SC, Myers B, Engelhardt J, Katz M, Baughn M, Platoshyn O, Marsala M, Watt A, Heyser CJ, Ard MC, De Muynck L, Daughrity LM, Swing DA, Tessarollo L, Jung CJ, Delpoux A, Utzschneider DT, Hedrick SM, de Jong PJ, Edbauer D, Van Damme P, Petrucelli L, Shaw CE, Bennett CF, Da Cruz S, Ravits J, Rigo F, Cleveland DW, Lagier-Tourenne C
Neuron 2016 May 4;90(3):535-50
Neuron 2016 May 4;90(3):535-50
Altered localization and functionality of TAR DNA Binding Protein 43 (TDP-43) in niemann- pick disease type C.
Dardis A, Zampieri S, Canterini S, Newell KL, Stuani C, Murrell JR, Ghetti B, Fiorenza MT, Bembi B, Buratti E
Acta neuropathologica communications 2016 May 18;4(1):52
Acta neuropathologica communications 2016 May 18;4(1):52
Poly-dipeptides encoded by the C9ORF72 repeats block global protein translation.
Kanekura K, Yagi T, Cammack AJ, Mahadevan J, Kuroda M, Harms MB, Miller TM, Urano F
Human molecular genetics 2016 May 1;25(9):1803-13
Human molecular genetics 2016 May 1;25(9):1803-13
T-Cell-Mediated Inflammatory Myopathies in HIV-Positive Individuals: A Histologic Study of 19 Cases.
Hiniker A, Daniels BH, Margeta M
Journal of neuropathology and experimental neurology 2016 Mar;75(3):239-45
Journal of neuropathology and experimental neurology 2016 Mar;75(3):239-45
Mechanistic Insights into Hsp104 Potentiation.
Torrente MP, Chuang E, Noll MM, Jackrel ME, Go MS, Shorter J
The Journal of biological chemistry 2016 Mar 4;291(10):5101-15
The Journal of biological chemistry 2016 Mar 4;291(10):5101-15
Loss of VPS13C Function in Autosomal-Recessive Parkinsonism Causes Mitochondrial Dysfunction and Increases PINK1/Parkin-Dependent Mitophagy.
Lesage S, Drouet V, Majounie E, Deramecourt V, Jacoupy M, Nicolas A, Cormier-Dequaire F, Hassoun SM, Pujol C, Ciura S, Erpapazoglou Z, Usenko T, Maurage CA, Sahbatou M, Liebau S, Ding J, Bilgic B, Emre M, Erginel-Unaltuna N, Guven G, Tison F, Tranchant C, Vidailhet M, Corvol JC, Krack P, Leutenegger AL, Nalls MA, Hernandez DG, Heutink P, Gibbs JR, Hardy J, Wood NW, Gasser T, Durr A, Deleuze JF, Tazir M, Destée A, Lohmann E, Kabashi E, Singleton A, Corti O, Brice A, French Parkinson's Disease Genetics Study (PDG), International Parkinson's Disease Genomics Consortium (IPDGC)
American journal of human genetics 2016 Mar 3;98(3):500-513
American journal of human genetics 2016 Mar 3;98(3):500-513
G3BP-Caprin1-USP10 complexes mediate stress granule condensation and associate with 40S subunits.
Kedersha N, Panas MD, Achorn CA, Lyons S, Tisdale S, Hickman T, Thomas M, Lieberman J, McInerney GM, Ivanov P, Anderson P
The Journal of cell biology 2016 Mar 28;212(7):845-60
The Journal of cell biology 2016 Mar 28;212(7):845-60
C9orf72 is required for proper macrophage and microglial function in mice.
O'Rourke JG, Bogdanik L, Yáñez A, Lall D, Wolf AJ, Muhammad AK, Ho R, Carmona S, Vit JP, Zarrow J, Kim KJ, Bell S, Harms MB, Miller TM, Dangler CA, Underhill DM, Goodridge HS, Lutz CM, Baloh RH
Science (New York, N.Y.) 2016 Mar 18;351(6279):1324-9
Science (New York, N.Y.) 2016 Mar 18;351(6279):1324-9
Mass spectrometric analysis of accumulated TDP-43 in amyotrophic lateral sclerosis brains.
Kametani F, Obi T, Shishido T, Akatsu H, Murayama S, Saito Y, Yoshida M, Hasegawa M
Scientific reports 2016 Mar 16;6:23281
Scientific reports 2016 Mar 16;6:23281
Phosphorylation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Truncated Casein Kinase 1δ Triggers Mislocalization and Accumulation of TDP-43.
Nonaka T, Suzuki G, Tanaka Y, Kametani F, Hirai S, Okado H, Miyashita T, Saitoe M, Akiyama H, Masai H, Hasegawa M
The Journal of biological chemistry 2016 Mar 11;291(11):5473-5483
The Journal of biological chemistry 2016 Mar 11;291(11):5473-5483
Motor neurone disease/amyotrophic lateral sclerosis associated with intermediate-length CAG repeat expansions in Ataxin-2 does not have 1C2-positive polyglutamine inclusions.
Highley JR, Lorente Pons A, Cooper-Knock J, Wharton SB, Ince PG, Shaw PJ, Wood J, Kirby J
Neuropathology and applied neurobiology 2016 Jun;42(4):377-89
Neuropathology and applied neurobiology 2016 Jun;42(4):377-89
An autopsied case of unclassifiable sporadic four-repeat tauopathy presenting with parkinsonism and speech disturbances.
Iwasaki Y, Mori K, Ito M, Tatsumi S, Mimuro M, Kuwano R, Hasegawa M, Yoshida M
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Jun;36(3):295-304
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Jun;36(3):295-304
Defining the spectrum of frontotemporal dementias associated with TARDBP mutations.
Caroppo P, Camuzat A, Guillot-Noel L, Thomas-Antérion C, Couratier P, Wong TH, Teichmann M, Golfier V, Auriacombe S, Belliard S, Laurent B, Lattante S, Millecamps S, Clot F, Dubois B, van Swieten JC, Brice A, Le Ber I
Neurology. Genetics 2016 Jun;2(3):e80
Neurology. Genetics 2016 Jun;2(3):e80
Misregulation of Alternative Splicing in a Mouse Model of Rett Syndrome.
Li R, Dong Q, Yuan X, Zeng X, Gao Y, Chiao C, Li H, Zhao X, Keles S, Wang Z, Chang Q
PLoS genetics 2016 Jun;12(6):e1006129
PLoS genetics 2016 Jun;12(6):e1006129
Phosphorylated TDP-43 becomes resistant to cleavage by calpain: A regulatory role for phosphorylation in TDP-43 pathology of ALS/FTLD.
Yamashita T, Teramoto S, Kwak S
Neuroscience research 2016 Jun;107:63-9
Neuroscience research 2016 Jun;107:63-9
Phenotypic characteristics of Alzheimer patients carrying an ABCA7 mutation.
Van den Bossche T, Sleegers K, Cuyvers E, Engelborghs S, Sieben A, De Roeck A, Van Cauwenberghe C, Vermeulen S, Van den Broeck M, Laureys A, Peeters K, Mattheijssens M, Vandenbulcke M, Vandenberghe R, Martin JJ, De Deyn PP, Cras P, Van Broeckhoven C, Belgian Neurology Consortium
Neurology 2016 Jun 7;86(23):2126-33
Neurology 2016 Jun 7;86(23):2126-33
The AMPA receptor antagonist perampanel robustly rescues amyotrophic lateral sclerosis (ALS) pathology in sporadic ALS model mice.
Akamatsu M, Yamashita T, Hirose N, Teramoto S, Kwak S
Scientific reports 2016 Jun 28;6:28649
Scientific reports 2016 Jun 28;6:28649
Heterogeneity of cerebral TDP-43 pathology in sporadic amyotrophic lateral sclerosis: Evidence for clinico-pathologic subtypes.
Takeuchi R, Tada M, Shiga A, Toyoshima Y, Konno T, Sato T, Nozaki H, Kato T, Horie M, Shimizu H, Takebayashi H, Onodera O, Nishizawa M, Kakita A, Takahashi H
Acta neuropathologica communications 2016 Jun 23;4(1):61
Acta neuropathologica communications 2016 Jun 23;4(1):61
A novel Drosophila model of TDP-43 proteinopathies: N-terminal sequences combined with the Q/N domain induce protein functional loss and locomotion defects.
Langellotti S, Romano V, Romano G, Klima R, Feiguin F, Cragnaz L, Romano M, Baralle FE
Disease models & mechanisms 2016 Jun 1;9(6):659-69
Disease models & mechanisms 2016 Jun 1;9(6):659-69
Argyrophilic Grain Disease: Demographics, Clinical, and Neuropathological Features From a Large Autopsy Study.
Rodriguez RD, Suemoto CK, Molina M, Nascimento CF, Leite RE, de Lucena Ferretti-Rebustini RE, Farfel JM, Heinsen H, Nitrini R, Ueda K, Pasqualucci CA, Jacob-Filho W, Yaffe K, Grinberg LT
Journal of neuropathology and experimental neurology 2016 Jul;75(7):628-35
Journal of neuropathology and experimental neurology 2016 Jul;75(7):628-35
Increased cytoplasmic TARDBP mRNA in affected spinal motor neurons in ALS caused by abnormal autoregulation of TDP-43.
Koyama A, Sugai A, Kato T, Ishihara T, Shiga A, Toyoshima Y, Koyama M, Konno T, Hirokawa S, Yokoseki A, Nishizawa M, Kakita A, Takahashi H, Onodera O
Nucleic acids research 2016 Jul 8;44(12):5820-36
Nucleic acids research 2016 Jul 8;44(12):5820-36
Distinct and shared functions of ALS-associated proteins TDP-43, FUS and TAF15 revealed by multisystem analyses.
Kapeli K, Pratt GA, Vu AQ, Hutt KR, Martinez FJ, Sundararaman B, Batra R, Freese P, Lambert NJ, Huelga SC, Chun SJ, Liang TY, Chang J, Donohue JP, Shiue L, Zhang J, Zhu H, Cambi F, Kasarskis E, Hoon S, Ares M Jr, Burge CB, Ravits J, Rigo F, Yeo GW
Nature communications 2016 Jul 5;7:12143
Nature communications 2016 Jul 5;7:12143
Global identification of hnRNP A1 binding sites for SSO-based splicing modulation.
Bruun GH, Doktor TK, Borch-Jensen J, Masuda A, Krainer AR, Ohno K, Andresen BS
BMC biology 2016 Jul 5;14:54
BMC biology 2016 Jul 5;14:54
Chronic Traumatic Encephalopathy Presenting as Alzheimer's Disease in a Retired Soccer Player.
Grinberg LT, Anghinah R, Nascimento CF, Amaro E, Leite RP, Martin Mda G, Naslavsky MS, Takada LT, Filho WJ, Pasqualucci CA, Nitrini R
Journal of Alzheimer's disease : JAD 2016 Jul 29;54(1):169-74
Journal of Alzheimer's disease : JAD 2016 Jul 29;54(1):169-74
Lentiviral vector-mediated overexpression of mutant ataxin-7 recapitulates SCA7 pathology and promotes accumulation of the FUS/TLS and MBNL1 RNA-binding proteins.
Alves S, Marais T, Biferi MG, Furling D, Marinello M, El Hachimi K, Cartier N, Ruberg M, Stevanin G, Brice A, Barkats M, Sittler A
Molecular neurodegeneration 2016 Jul 28;11(1):58
Molecular neurodegeneration 2016 Jul 28;11(1):58
MTHFSD and DDX58 are novel RNA-binding proteins abnormally regulated in amyotrophic lateral sclerosis.
MacNair L, Xiao S, Miletic D, Ghani M, Julien JP, Keith J, Zinman L, Rogaeva E, Robertson J
Brain : a journal of neurology 2016 Jan;139(Pt 1):86-100
Brain : a journal of neurology 2016 Jan;139(Pt 1):86-100
Mutations in HSPB8 causing a new phenotype of distal myopathy and motor neuropathy.
Ghaoui R, Palmio J, Brewer J, Lek M, Needham M, Evilä A, Hackman P, Jonson PH, Penttilä S, Vihola A, Huovinen S, Lindfors M, Davis RL, Waddell L, Kaur S, Yiannikas C, North K, Clarke N, MacArthur DG, Sue CM, Udd B
Neurology 2016 Jan 26;86(4):391-8
Neurology 2016 Jan 26;86(4):391-8
TDP-43 loss of function increases TFEB activity and blocks autophagosome-lysosome fusion.
Xia Q, Wang H, Hao Z, Fu C, Hu Q, Gao F, Ren H, Chen D, Han J, Ying Z, Wang G
The EMBO journal 2016 Jan 18;35(2):121-42
The EMBO journal 2016 Jan 18;35(2):121-42
CUL2-mediated clearance of misfolded TDP-43 is paradoxically affected by VHL in oligodendrocytes in ALS.
Uchida T, Tamaki Y, Ayaki T, Shodai A, Kaji S, Morimura T, Banno Y, Nishitsuji K, Sakashita N, Maki T, Yamashita H, Ito H, Takahashi R, Urushitani M
Scientific reports 2016 Jan 11;6:19118
Scientific reports 2016 Jan 11;6:19118
Intravenous injection of l-BMAA induces a rat model with comprehensive characteristics of amyotrophic lateral sclerosis/Parkinson-dementia complex.
Tian KW, Jiang H, Wang BB, Zhang F, Han S
Toxicology research 2016 Jan 1;5(1):79-96
Toxicology research 2016 Jan 1;5(1):79-96
An autopsy case of frontotemporal lobar degeneration with the appearance of fused in sarcoma inclusions (basophilic inclusion body disease) clinically presenting corticobasal syndrome.
Matsumoto A, Suzuki H, Fukatsu R, Shimizu H, Suzuki Y, Hisanaga K
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Feb;36(1):77-87
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Feb;36(1):77-87
Clinicopathological description of two cases with SQSTM1 gene mutation associated with frontotemporal dementia.
Kovacs GG, van der Zee J, Hort J, Kristoferitsch W, Leitha T, Höftberger R, Ströbel T, Van Broeckhoven C, Matej R
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Feb;36(1):27-38
Neuropathology : official journal of the Japanese Society of Neuropathology 2016 Feb;36(1):27-38
Clinical features of TBK1 carriers compared with C9orf72, GRN and non-mutation carriers in a Belgian cohort.
Van Mossevelde S, van der Zee J, Gijselinck I, Engelborghs S, Sieben A, Van Langenhove T, De Bleecker J, Baets J, Vandenbulcke M, Van Laere K, Ceyssens S, Van den Broeck M, Peeters K, Mattheijssens M, Cras P, Vandenberghe R, De Jonghe P, Martin JJ, De Deyn PP, Cruts M, Van Broeckhoven C, Belgian Neurology consortium
Brain : a journal of neurology 2016 Feb;139(Pt 2):452-67
Brain : a journal of neurology 2016 Feb;139(Pt 2):452-67
Serine 403-phosphorylated p62/SQSTM1 immunoreactivity in inclusions of neurodegenerative diseases.
Kurosawa M, Matsumoto G, Sumikura H, Hatsuta H, Murayama S, Sakurai T, Shimogori T, Hattori N, Nukina N
Neuroscience research 2016 Feb;103:64-70
Neuroscience research 2016 Feb;103:64-70
Diagnostically important muscle pathology in DNAJB6 mutated LGMD1D.
Sandell S, Huovinen S, Palmio J, Raheem O, Lindfors M, Zhao F, Haapasalo H, Udd B
Acta neuropathologica communications 2016 Feb 5;4:9
Acta neuropathologica communications 2016 Feb 5;4:9
Melatonin enhances arsenic trioxide-induced cell death via sustained upregulation of Redd1 expression in breast cancer cells.
Yun SM, Woo SH, Oh ST, Hong SE, Choe TB, Ye SK, Kim EK, Seong MK, Kim HA, Noh WC, Lee JK, Jin HO, Lee YH, Park IC
Molecular and cellular endocrinology 2016 Feb 15;422:64-73
Molecular and cellular endocrinology 2016 Feb 15;422:64-73
In vitro prion-like behaviour of TDP-43 in ALS.
Smethurst P, Newcombe J, Troakes C, Simone R, Chen YR, Patani R, Sidle K
Neurobiology of disease 2016 Dec;96:236-247
Neurobiology of disease 2016 Dec;96:236-247
Timing and significance of pathological features in C9orf72 expansion-associated frontotemporal dementia.
Vatsavayai SC, Yoon SJ, Gardner RC, Gendron TF, Vargas JN, Trujillo A, Pribadi M, Phillips JJ, Gaus SE, Hixson JD, Garcia PA, Rabinovici GD, Coppola G, Geschwind DH, Petrucelli L, Miller BL, Seeley WW
Brain : a journal of neurology 2016 Dec;139(Pt 12):3202-3216
Brain : a journal of neurology 2016 Dec;139(Pt 12):3202-3216
Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model.
LaClair KD, Donde A, Ling JP, Jeong YH, Chhabra R, Martin LJ, Wong PC
Acta neuropathologica 2016 Dec;132(6):859-873
Acta neuropathologica 2016 Dec;132(6):859-873
Imbalance of mitochondrial dynamics in Drosophila models of amyotrophic lateral sclerosis.
Altanbyek V, Cha SJ, Kang GU, Im DS, Lee S, Kim HJ, Kim K
Biochemical and biophysical research communications 2016 Dec 9;481(3-4):259-264
Biochemical and biophysical research communications 2016 Dec 9;481(3-4):259-264
Nuclear bodies reorganize during myogenesis in vitro and are differentially disrupted by expression of FSHD-associated DUX4.
Homma S, Beermann ML, Yu B, Boyce FM, Miller JB
Skeletal muscle 2016 Dec 1;6(1):42
Skeletal muscle 2016 Dec 1;6(1):42
Marked Involvement of the Striatal Efferent System in TAR DNA-Binding Protein 43 kDa-Related Frontotemporal Lobar Degeneration and Amyotrophic Lateral Sclerosis.
Riku Y, Watanabe H, Yoshida M, Mimuro M, Iwasaki Y, Masuda M, Ishigaki S, Katsuno M, Sobue G
Journal of neuropathology and experimental neurology 2016 Aug;75(8):801-811
Journal of neuropathology and experimental neurology 2016 Aug;75(8):801-811
The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity.
Wang W, Wang L, Lu J, Siedlak SL, Fujioka H, Liang J, Jiang S, Ma X, Jiang Z, da Rocha EL, Sheng M, Choi H, Lerou PH, Li H, Wang X
Nature medicine 2016 Aug;22(8):869-78
Nature medicine 2016 Aug;22(8):869-78
Severe muscle wasting and denervation in mice lacking the RNA-binding protein ZFP106.
Anderson DM, Cannavino J, Li H, Anderson KM, Nelson BR, McAnally J, Bezprozvannaya S, Liu Y, Lin W, Liu N, Bassel-Duby R, Olson EN
Proceedings of the National Academy of Sciences of the United States of America 2016 Aug 2;113(31):E4494-503
Proceedings of the National Academy of Sciences of the United States of America 2016 Aug 2;113(31):E4494-503
A transgenic mouse expressing CHMP2Bintron5 mutant in neurons develops histological and behavioural features of amyotrophic lateral sclerosis and frontotemporal dementia.
Vernay A, Therreau L, Blot B, Risson V, Dirrig-Grosch S, Waegaert R, Lequeu T, Sellal F, Schaeffer L, Sadoul R, Loeffler JP, René F
Human molecular genetics 2016 Aug 1;25(15):3341-3360
Human molecular genetics 2016 Aug 1;25(15):3341-3360
Neurodegeneration in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9orf72 is linked to TDP-43 pathology and not associated with aggregated forms of dipeptide repeat proteins.
Davidson Y, Robinson AC, Liu X, Wu D, Troakes C, Rollinson S, Masuda-Suzukake M, Suzuki G, Nonaka T, Shi J, Tian J, Hamdalla H, Ealing J, Richardson A, Jones M, Pickering-Brown S, Snowden JS, Hasegawa M, Mann DM
Neuropathology and applied neurobiology 2016 Apr;42(3):242-54
Neuropathology and applied neurobiology 2016 Apr;42(3):242-54
Multiple sclerosis masquerading as Alzheimer-type dementia: Clinical, radiological and pathological findings.
Tobin WO, Popescu BF, Lowe V, Pirko I, Parisi JE, Kantarci K, Fields JA, Bruns MB, Boeve BF, Lucchinetti CF
Multiple sclerosis (Houndmills, Basingstoke, England) 2016 Apr;22(5):698-704
Multiple sclerosis (Houndmills, Basingstoke, England) 2016 Apr;22(5):698-704
Targeting TDP-43 phosphorylation by Casein Kinase-1δ inhibitors: a novel strategy for the treatment of frontotemporal dementia.
Alquezar C, Salado IG, de la Encarnación A, Pérez DI, Moreno F, Gil C, de Munain AL, Martínez A, Martín-Requero Á
Molecular neurodegeneration 2016 Apr 30;11(1):36
Molecular neurodegeneration 2016 Apr 30;11(1):36
ESCRT-0 dysfunction compromises autophagic degradation of protein aggregates and facilitates ER stress-mediated neurodegeneration via apoptotic and necroptotic pathways.
Oshima R, Hasegawa T, Tamai K, Sugeno N, Yoshida S, Kobayashi J, Kikuchi A, Baba T, Futatsugi A, Sato I, Satoh K, Takeda A, Aoki M, Tanaka N
Scientific reports 2016 Apr 26;6:24997
Scientific reports 2016 Apr 26;6:24997
Templated Aggregation of TAR DNA-binding Protein of 43 kDa (TDP-43) by Seeding with TDP-43 Peptide Fibrils.
Shimonaka S, Nonaka T, Suzuki G, Hisanaga S, Hasegawa M
The Journal of biological chemistry 2016 Apr 22;291(17):8896-907
The Journal of biological chemistry 2016 Apr 22;291(17):8896-907
ALS patients with ability to communicate after long-term mechanical ventilation have confined degeneration to the motor neuron system.
Mochizuki Y, Hayashi K, Nakayama Y, Shimizu T, Kamide M, Ogino M, Komori T, Hasegawa M, Isozaki E, Nakano I
Journal of the neurological sciences 2016 Apr 15;363:245-8
Journal of the neurological sciences 2016 Apr 15;363:245-8
ALS-linked protein disulfide isomerase variants cause motor dysfunction.
Woehlbier U, Colombo A, Saaranen MJ, Pérez V, Ojeda J, Bustos FJ, Andreu CI, Torres M, Valenzuela V, Medinas DB, Rozas P, Vidal RL, Lopez-Gonzalez R, Salameh J, Fernandez-Collemann S, Muñoz N, Matus S, Armisen R, Sagredo A, Palma K, Irrazabal T, Almeida S, Gonzalez-Perez P, Campero M, Gao FB, Henny P, van Zundert B, Ruddock LW, Concha ML, Henriquez JP, Brown RH, Hetz C
The EMBO journal 2016 Apr 15;35(8):845-65
The EMBO journal 2016 Apr 15;35(8):845-65
Miro1 deficiency in amyotrophic lateral sclerosis.
Zhang F, Wang W, Siedlak SL, Liu Y, Liu J, Jiang K, Perry G, Zhu X, Wang X
Frontiers in aging neuroscience 2015;7:100
Frontiers in aging neuroscience 2015;7:100
Progressive supranuclear palsy in a family with TDP-43 pathology.
Kertesz A, Finger E, Murrell J, Chertkow H, Ang LC, Baker M, Ravenscroft T, Rademakers R, Munoz DG
Neurocase 2015;21(2):178-84
Neurocase 2015;21(2):178-84
AAV9 supports wide-scale transduction of the CNS and TDP-43 disease modeling in adult rats.
Jackson KL, Dayton RD, Klein RL
Molecular therapy. Methods & clinical development 2015;2:15036
Molecular therapy. Methods & clinical development 2015;2:15036
Identification of linc-NeD125, a novel long non coding RNA that hosts miR-125b-1 and negatively controls proliferation of human neuroblastoma cells.
Bevilacqua V, Gioia U, Di Carlo V, Tortorelli AF, Colombo T, Bozzoni I, Laneve P, Caffarelli E
RNA biology 2015;12(12):1323-37
RNA biology 2015;12(12):1323-37
Proteins with Intrinsically Disordered Domains Are Preferentially Recruited to Polyglutamine Aggregates.
Wear MP, Kryndushkin D, O'Meally R, Sonnenberg JL, Cole RN, Shewmaker FP
PloS one 2015;10(8):e0136362
PloS one 2015;10(8):e0136362
Saccadic Palsy following Cardiac Surgery: Possible Role of Perineuronal Nets.
Eggers SD, Horn AK, Roeber S, Härtig W, Nair G, Reich DS, Leigh RJ
PloS one 2015;10(7):e0132075
PloS one 2015;10(7):e0132075
A novel triple repeat mutant tau transgenic model that mimics aspects of pick's disease and fronto-temporal tauopathies.
Rockenstein E, Overk CR, Ubhi K, Mante M, Patrick C, Adame A, Bisquert A, Trejo-Morales M, Spencer B, Masliah E
PloS one 2015;10(3):e0121570
PloS one 2015;10(3):e0121570
TDP-43 Inhibits NF-κB Activity by Blocking p65 Nuclear Translocation.
Zhu J, Cynader MS, Jia W
PloS one 2015;10(11):e0142296
PloS one 2015;10(11):e0142296
Inflammation Induces TDP-43 Mislocalization and Aggregation.
Correia AS, Patel P, Dutta K, Julien JP
PloS one 2015;10(10):e0140248
PloS one 2015;10(10):e0140248
Spinocerebellar ataxia type 36 exists in diverse populations and can be caused by a short hexanucleotide GGCCTG repeat expansion.
Obayashi M, Stevanin G, Synofzik M, Monin ML, Duyckaerts C, Sato N, Streichenberger N, Vighetto A, Desestret V, Tesson C, Wichmann HE, Illig T, Huttenlocher J, Kita Y, Izumi Y, Mizusawa H, Schöls L, Klopstock T, Brice A, Ishikawa K, Dürr A
Journal of neurology, neurosurgery, and psychiatry 2015 Sep;86(9):986-95
Journal of neurology, neurosurgery, and psychiatry 2015 Sep;86(9):986-95
C9orf72 ablation in mice does not cause motor neuron degeneration or motor deficits.
Koppers M, Blokhuis AM, Westeneng HJ, Terpstra ML, Zundel CA, Vieira de Sá R, Schellevis RD, Waite AJ, Blake DJ, Veldink JH, van den Berg LH, Pasterkamp RJ
Annals of neurology 2015 Sep;78(3):426-38
Annals of neurology 2015 Sep;78(3):426-38
Exposure to ALS-FTD-CSF generates TDP-43 aggregates in glioblastoma cells through exosomes and TNTs-like structure.
Ding X, Ma M, Teng J, Teng RK, Zhou S, Yin J, Fonkem E, Huang JH, Wu E, Wang X
Oncotarget 2015 Sep 15;6(27):24178-91
Oncotarget 2015 Sep 15;6(27):24178-91
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Chou CC, Alexeeva OM, Yamada S, Pribadi A, Zhang Y, Mo B, Williams KR, Zarnescu DC, Rossoll W
Human molecular genetics 2015 Sep 15;24(18):5154-73
Human molecular genetics 2015 Sep 15;24(18):5154-73
Ubiquilin-2 drives NF-κB activity and cytosolic TDP-43 aggregation in neuronal cells.
Picher-Martel V, Dutta K, Phaneuf D, Sobue G, Julien JP
Molecular brain 2015 Oct 31;8(1):71
Molecular brain 2015 Oct 31;8(1):71
Validating novel tau positron emission tomography tracer [F-18]-AV-1451 (T807) on postmortem brain tissue.
Marquié M, Normandin MD, Vanderburg CR, Costantino IM, Bien EA, Rycyna LG, Klunk WE, Mathis CA, Ikonomovic MD, Debnath ML, Vasdev N, Dickerson BC, Gomperts SN, Growdon JH, Johnson KA, Frosch MP, Hyman BT, Gómez-Isla T
Annals of neurology 2015 Nov;78(5):787-800
Annals of neurology 2015 Nov;78(5):787-800
Stress Granules Modulate SYK to Cause Microglial Cell Dysfunction in Alzheimer's Disease.
Ghosh S, Geahlen RL
EBioMedicine 2015 Nov;2(11):1785-98
EBioMedicine 2015 Nov;2(11):1785-98
Early maturation and distinct tau pathology in induced pluripotent stem cell-derived neurons from patients with MAPT mutations.
Iovino M, Agathou S, González-Rueda A, Del Castillo Velasco-Herrera M, Borroni B, Alberici A, Lynch T, O'Dowd S, Geti I, Gaffney D, Vallier L, Paulsen O, Káradóttir RT, Spillantini MG
Brain : a journal of neurology 2015 Nov;138(Pt 11):3345-59
Brain : a journal of neurology 2015 Nov;138(Pt 11):3345-59
Short-term suppression of A315T mutant human TDP-43 expression improves functional deficits in a novel inducible transgenic mouse model of FTLD-TDP and ALS.
Ke YD, van Hummel A, Stevens CH, Gladbach A, Ippati S, Bi M, Lee WS, Krüger S, van der Hoven J, Volkerling A, Bongers A, Halliday G, Haass NK, Kiernan M, Delerue F, Ittner LM
Acta neuropathologica 2015 Nov;130(5):661-78
Acta neuropathologica 2015 Nov;130(5):661-78
Dysregulated miRNA biogenesis downstream of cellular stress and ALS-causing mutations: a new mechanism for ALS.
Emde A, Eitan C, Liou LL, Libby RT, Rivkin N, Magen I, Reichenstein I, Oppenheim H, Eilam R, Silvestroni A, Alajajian B, Ben-Dov IZ, Aebischer J, Savidor A, Levin Y, Sons R, Hammond SM, Ravits JM, Möller T, Hornstein E
The EMBO journal 2015 Nov 3;34(21):2633-51
The EMBO journal 2015 Nov 3;34(21):2633-51
TDP-43 is intercellularly transmitted across axon terminals.
Feiler MS, Strobel B, Freischmidt A, Helferich AM, Kappel J, Brewer BM, Li D, Thal DR, Walther P, Ludolph AC, Danzer KM, Weishaupt JH
The Journal of cell biology 2015 Nov 23;211(4):897-911
The Journal of cell biology 2015 Nov 23;211(4):897-911
Amyotrophic lateral sclerosis-associated mutant profilin 1 increases dendritic arborisation and spine formation in primary hippocampal neurons.
Brettle M, Suchowerska AK, Chua SW, Ittner LM, Fath T
Neuroscience letters 2015 Nov 16;609:223-8
Neuroscience letters 2015 Nov 16;609:223-8
Neuropathologic features of suicide victims who presented with acute poststroke depression: significance of association with neurodegenerative disorders.
Nishida N, Hata Y, Yoshida K, Kinoshita K
Journal of neuropathology and experimental neurology 2015 May;74(5):401-10
Journal of neuropathology and experimental neurology 2015 May;74(5):401-10
Analysis of β-N-methylamino-L-alanine (L-BMAA) neurotoxicity in rat cerebellum.
Muñoz-Sáez E, de Munck García E, Arahuetes Portero RM, Martínez A, Solas Alados MT, Miguel BG
Neurotoxicology 2015 May;48:192-205
Neurotoxicology 2015 May;48:192-205
Primary fibroblasts cultures reveal TDP-43 abnormalities in amyotrophic lateral sclerosis patients with and without SOD1 mutations.
Sabatelli M, Zollino M, Conte A, Del Grande A, Marangi G, Lucchini M, Mirabella M, Romano A, Piacentini R, Bisogni G, Lattante S, Luigetti M, Rossini PM, Moncada A
Neurobiology of aging 2015 May;36(5):2005.e5-2005.e13
Neurobiology of aging 2015 May;36(5):2005.e5-2005.e13
A Peruvian family with a novel PARK2 mutation: Clinical and pathological characteristics.
Cornejo-Olivas MR, Torres L, Mata IF, Mazzetti P, Rivas D, Cosentino C, Inca-Martinez M, Cuba JM, Zabetian CP, Leverenz JB
Parkinsonism & related disorders 2015 May;21(5):444-8
Parkinsonism & related disorders 2015 May;21(5):444-8
Haploinsufficiency of TBK1 causes familial ALS and fronto-temporal dementia.
Freischmidt A, Wieland T, Richter B, Ruf W, Schaeffer V, Müller K, Marroquin N, Nordin F, Hübers A, Weydt P, Pinto S, Press R, Millecamps S, Molko N, Bernard E, Desnuelle C, Soriani MH, Dorst J, Graf E, Nordström U, Feiler MS, Putz S, Boeckers TM, Meyer T, Winkler AS, Winkelman J, de Carvalho M, Thal DR, Otto M, Brännström T, Volk AE, Kursula P, Danzer KM, Lichtner P, Dikic I, Meitinger T, Ludolph AC, Strom TM, Andersen PM, Weishaupt JH
Nature neuroscience 2015 May;18(5):631-6
Nature neuroscience 2015 May;18(5):631-6
Proteomic analyses reveal that loss of TDP-43 affects RNA processing and intracellular transport.
Štalekar M, Yin X, Rebolj K, Darovic S, Troakes C, Mayr M, Shaw CE, Rogelj B
Neuroscience 2015 May 7;293:157-70
Neuroscience 2015 May 7;293:157-70
Nucleolar stress and impaired stress granule formation contribute to C9orf72 RAN translation-induced cytotoxicity.
Tao Z, Wang H, Xia Q, Li K, Li K, Jiang X, Xu G, Wang G, Ying Z
Human molecular genetics 2015 May 1;24(9):2426-41
Human molecular genetics 2015 May 1;24(9):2426-41
Nuclear accumulation of mRNAs underlies G4C2-repeat-induced translational repression in a cellular model of C9orf72 ALS.
Rossi S, Serrano A, Gerbino V, Giorgi A, Di Francesco L, Nencini M, Bozzo F, Schininà ME, Bagni C, Cestra G, Carrì MT, Achsel T, Cozzolino M
Journal of cell science 2015 May 1;128(9):1787-99
Journal of cell science 2015 May 1;128(9):1787-99
Defining neurodegeneration on Guam by targeted genomic sequencing.
Steele JC, Guella I, Szu-Tu C, Lin MK, Thompson C, Evans DM, Sherman HE, Vilariño-Güell C, Gwinn K, Morris H, Dickson DW, Farrer MJ
Annals of neurology 2015 Mar;77(3):458-68
Annals of neurology 2015 Mar;77(3):458-68
TARDBP pathogenic mutations increase cytoplasmic translocation of TDP-43 and cause reduction of endoplasmic reticulum Ca²⁺ signaling in motor neurons.
Mutihac R, Alegre-Abarrategui J, Gordon D, Farrimond L, Yamasaki-Mann M, Talbot K, Wade-Martins R
Neurobiology of disease 2015 Mar;75:64-77
Neurobiology of disease 2015 Mar;75:64-77
TDP-43 modification in the hSOD1(G93A) amyotrophic lateral sclerosis mouse model.
Cai M, Lee KW, Choi SM, Yang EJ
Neurological research 2015 Mar;37(3):253-62
Neurological research 2015 Mar;37(3):253-62
Increasing progranulin levels and blockade of the ERK1/2 pathway: upstream and downstream strategies for the treatment of progranulin deficient frontotemporal dementia.
Alquezar C, Esteras N, de la Encarnación A, Moreno F, López de Munain A, Martín-Requero Á
European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology 2015 Mar;25(3):386-403
European neuropsychopharmacology : the journal of the European College of Neuropsychopharmacology 2015 Mar;25(3):386-403
Pathogenic Ubqln2 gains toxic properties to induce neuron death.
Wu Q, Liu M, Huang C, Liu X, Huang B, Li N, Zhou H, Xia XG
Acta neuropathologica 2015 Mar;129(3):417-28
Acta neuropathologica 2015 Mar;129(3):417-28
Phosphorylation of hnRNP K by cyclin-dependent kinase 2 controls cytosolic accumulation of TDP-43.
Moujalled D, James JL, Yang S, Zhang K, Duncan C, Moujalled DM, Parker SJ, Caragounis A, Lidgerwood G, Turner BJ, Atkin JD, Grubman A, Liddell JR, Proepper C, Boeckers TM, Kanninen KM, Blair I, Crouch PJ, White AR
Human molecular genetics 2015 Mar 15;24(6):1655-69
Human molecular genetics 2015 Mar 15;24(6):1655-69
Characterization of the dipeptide repeat protein in the molecular pathogenesis of c9FTD/ALS.
Yamakawa M, Ito D, Honda T, Kubo K, Noda M, Nakajima K, Suzuki N
Human molecular genetics 2015 Mar 15;24(6):1630-45
Human molecular genetics 2015 Mar 15;24(6):1630-45
Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs.
Suzuki H, Shibagaki Y, Hattori S, Matsuoka M
Human molecular genetics 2015 Mar 15;24(6):1513-27
Human molecular genetics 2015 Mar 15;24(6):1513-27
Hippocampal sclerosis in the parkinsonism-dementia complex of Guam: quantitative examination of neurons, neurofibrillary tangles, and TDP-43 immunoreactivity in CA1.
Oyanagi K, Yamazaki M, Hashimoto T, Asakawa M, Wakabayashi K, Takahashi H
Neuropathology : official journal of the Japanese Society of Neuropathology 2015 Jun;35(3):224-35
Neuropathology : official journal of the Japanese Society of Neuropathology 2015 Jun;35(3):224-35
Minimal change multiple system atrophy: an aggressive variant?
Ling H, Asi YT, Petrovic IN, Ahmed Z, Prashanth LK, Hazrati LN, Nishizawa M, Ozawa T, Lang A, Lees AJ, Revesz T, Holton JL
Movement disorders : official journal of the Movement Disorder Society 2015 Jun;30(7):960-7
Movement disorders : official journal of the Movement Disorder Society 2015 Jun;30(7):960-7
Neurodegeneration. C9ORF72 repeat expansions in mice cause TDP-43 pathology, neuronal loss, and behavioral deficits.
Chew J, Gendron TF, Prudencio M, Sasaguri H, Zhang YJ, Castanedes-Casey M, Lee CW, Jansen-West K, Kurti A, Murray ME, Bieniek KF, Bauer PO, Whitelaw EC, Rousseau L, Stankowski JN, Stetler C, Daughrity LM, Perkerson EA, Desaro P, Johnston A, Overstreet K, Edbauer D, Rademakers R, Boylan KB, Dickson DW, Fryer JD, Petrucelli L
Science (New York, N.Y.) 2015 Jun 5;348(6239):1151-4
Science (New York, N.Y.) 2015 Jun 5;348(6239):1151-4
Wild type human TDP-43 potentiates ALS-linked mutant TDP-43 driven progressive motor and cortical neuron degeneration with pathological features of ALS.
Mitchell JC, Constable R, So E, Vance C, Scotter E, Glover L, Hortobagyi T, Arnold ES, Ling SC, McAlonis M, Da Cruz S, Polymenidou M, Tessarolo L, Cleveland DW, Shaw CE
Acta neuropathologica communications 2015 Jun 25;3:36
Acta neuropathologica communications 2015 Jun 25;3:36
Oxr1 improves pathogenic cellular features of ALS-associated FUS and TDP-43 mutations.
Finelli MJ, Liu KX, Wu Y, Oliver PL, Davies KE
Human molecular genetics 2015 Jun 15;24(12):3529-44
Human molecular genetics 2015 Jun 15;24(12):3529-44
Neurodegeneration in C. elegans models of ALS requires TIR-1/Sarm1 immune pathway activation in neurons.
Vérièpe J, Fossouo L, Parker JA
Nature communications 2015 Jun 10;6:7319
Nature communications 2015 Jun 10;6:7319
Antisense RNA foci in the motor neurons of C9ORF72-ALS patients are associated with TDP-43 proteinopathy.
Cooper-Knock J, Higginbottom A, Stopford MJ, Highley JR, Ince PG, Wharton SB, Pickering-Brown S, Kirby J, Hautbergue GM, Shaw PJ
Acta neuropathologica 2015 Jul;130(1):63-75
Acta neuropathologica 2015 Jul;130(1):63-75
Low molecular weight species of TDP-43 generated by abnormal splicing form inclusions in amyotrophic lateral sclerosis and result in motor neuron death.
Xiao S, Sanelli T, Chiang H, Sun Y, Chakrabartty A, Keith J, Rogaeva E, Zinman L, Robertson J
Acta neuropathologica 2015 Jul;130(1):49-61
Acta neuropathologica 2015 Jul;130(1):49-61
Neuropathological assessments of the pathology in frontotemporal lobar degeneration with TDP43-positive inclusions: an inter-laboratory study by the BrainNet Europe consortium.
Alafuzoff I, Pikkarainen M, Neumann M, Arzberger T, Al-Sarraj S, Bodi I, Bogdanovic N, Bugiani O, Ferrer I, Gelpi E, Gentleman S, Giaccone G, Graeber MB, Hortobagyi T, Ince PG, Ironside JW, Kavantzas N, King A, Korkolopoulou P, Kovács GG, Meyronet D, Monoranu C, Nilsson T, Parchi P, Patsouris E, Revesz T, Roggendorf W, Rozemuller A, Seilhean D, Streichenberger N, Thal DR, Wharton SB, Kretzschmar H
Journal of neural transmission (Vienna, Austria : 1996) 2015 Jul;122(7):957-72
Journal of neural transmission (Vienna, Austria : 1996) 2015 Jul;122(7):957-72
Spread of pathology in amyotrophic lateral sclerosis: assessment of phosphorylated TDP-43 along axonal pathways.
Fatima M, Tan R, Halliday GM, Kril JJ
Acta neuropathologica communications 2015 Jul 28;3:47
Acta neuropathologica communications 2015 Jul 28;3:47
Complete loss of the DNAJB6 G/F domain and novel missense mutations cause distal-onset DNAJB6 myopathy.
Ruggieri A, Brancati F, Zanotti S, Maggi L, Pasanisi MB, Saredi S, Terracciano C, Antozzi C, D Apice MR, Sangiuolo F, Novelli G, Marshall CR, Scherer SW, Morandi L, Federici L, Massa R, Mora M, Minassian BA
Acta neuropathologica communications 2015 Jul 25;3:44
Acta neuropathologica communications 2015 Jul 25;3:44
4-Hydroxynonenal induces persistent insolubilization of TDP-43 and alters its intracellular localization.
Kabuta C, Kono K, Wada K, Kabuta T
Biochemical and biophysical research communications 2015 Jul 17-24;463(1-2):82-7
Biochemical and biophysical research communications 2015 Jul 17-24;463(1-2):82-7
Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia.
Ziskin JL, Greicius MD, Zhu W, Okumu AN, Adams CM, Plowey ED
Acta neuropathologica communications 2015 Jul 10;3:43
Acta neuropathologica communications 2015 Jul 10;3:43
Hippocampal sclerosis in Lewy body disease is a TDP-43 proteinopathy similar to FTLD-TDP Type A.
Aoki N, Murray ME, Ogaki K, Fujioka S, Rutherford NJ, Rademakers R, Ross OA, Dickson DW
Acta neuropathologica 2015 Jan;129(1):53-64
Acta neuropathologica 2015 Jan;129(1):53-64
An acetylation switch controls TDP-43 function and aggregation propensity.
Cohen TJ, Hwang AW, Restrepo CR, Yuan CX, Trojanowski JQ, Lee VM
Nature communications 2015 Jan 5;6:5845
Nature communications 2015 Jan 5;6:5845
The cleavage pattern of TDP-43 determines its rate of clearance and cytotoxicity.
Li Q, Yokoshi M, Okada H, Kawahara Y
Nature communications 2015 Jan 29;6:6183
Nature communications 2015 Jan 29;6:6183
TDP-43 loss of cellular function through aggregation requires additional structural determinants beyond its C-terminal Q/N prion-like domain.
Budini M, Romano V, Quadri Z, Buratti E, Baralle FE
Human molecular genetics 2015 Jan 1;24(1):9-20
Human molecular genetics 2015 Jan 1;24(1):9-20
Changes in TDP-43 expression in development, aging, and in the neurofilament light protein knockout mouse.
Liu Y, Atkinson RA, Fernandez-Martos CM, Kirkcaldie MT, Cui H, Vickers JC, King AE
Neurobiology of aging 2015 Feb;36(2):1151-9
Neurobiology of aging 2015 Feb;36(2):1151-9
Atypical association of semantic dementia, corticobasal syndrome, and 4R tauopathy.
Clerc MT, Deprez M, Leuba G, Lhermitte B, Lopez U, von Gunten A
Neurocase 2015 Feb;21(1):1-15
Neurocase 2015 Feb;21(1):1-15
Expression of FSHD-related DUX4-FL alters proteostasis and induces TDP-43 aggregation.
Homma S, Beermann ML, Boyce FM, Miller JB
Annals of clinical and translational neurology 2015 Feb;2(2):151-66
Annals of clinical and translational neurology 2015 Feb;2(2):151-66
Novel atomic force microscopy based biopanning for isolation of morphology specific reagents against TDP-43 variants in amyotrophic lateral sclerosis.
Williams SM, Venkataraman L, Tian H, Khan G, Harris BT, Sierks MR
Journal of visualized experiments : JoVE 2015 Feb 12;(96)
Journal of visualized experiments : JoVE 2015 Feb 12;(96)
Opposing roles of p38 and JNK in a Drosophila model of TDP-43 proteinopathy reveal oxidative stress and innate immunity as pathogenic components of neurodegeneration.
Zhan L, Xie Q, Tibbetts RS
Human molecular genetics 2015 Feb 1;24(3):757-72
Human molecular genetics 2015 Feb 1;24(3):757-72
Primary chronic traumatic encephalopathy in an older patient with late-onset AD phenotype.
Fredericks CA, Koestler M, Seeley W, Miller B, Boxer A, Grinberg LT
Neurology. Clinical practice 2015 Dec;5(6):475-479
Neurology. Clinical practice 2015 Dec;5(6):475-479
Pathological features of preclinical or early clinical stages of corticobasal degeneration: a comparison with advanced cases.
Nishida N, Yoshida K, Hata Y, Arai Y, Kinoshita K
Neuropathology and applied neurobiology 2015 Dec;41(7):893-905
Neuropathology and applied neurobiology 2015 Dec;41(7):893-905
From transcriptomic to protein level changes in TDP-43 and FUS loss-of-function cell models.
Colombrita C, Onesto E, Buratti E, de la Grange P, Gumina V, Baralle FE, Silani V, Ratti A
Biochimica et biophysica acta 2015 Dec;1849(12):1398-410
Biochimica et biophysica acta 2015 Dec;1849(12):1398-410
Quantitative analysis and clinico-pathological correlations of different dipeptide repeat protein pathologies in C9ORF72 mutation carriers.
Mackenzie IR, Frick P, Grässer FA, Gendron TF, Petrucelli L, Cashman NR, Edbauer D, Kremmer E, Prudlo J, Troost D, Neumann M
Acta neuropathologica 2015 Dec;130(6):845-61
Acta neuropathologica 2015 Dec;130(6):845-61
Human C9ORF72 Hexanucleotide Expansion Reproduces RNA Foci and Dipeptide Repeat Proteins but Not Neurodegeneration in BAC Transgenic Mice.
Peters OM, Cabrera GT, Tran H, Gendron TF, McKeon JE, Metterville J, Weiss A, Wightman N, Salameh J, Kim J, Sun H, Boylan KB, Dickson D, Kennedy Z, Lin Z, Zhang YJ, Daughrity L, Jung C, Gao FB, Sapp PC, Horvitz HR, Bosco DA, Brown SP, de Jong P, Petrucelli L, Mueller C, Brown RH Jr
Neuron 2015 Dec 2;88(5):902-909
Neuron 2015 Dec 2;88(5):902-909
C9orf72 BAC Transgenic Mice Display Typical Pathologic Features of ALS/FTD.
O'Rourke JG, Bogdanik L, Muhammad AKMG, Gendron TF, Kim KJ, Austin A, Cady J, Liu EY, Zarrow J, Grant S, Ho R, Bell S, Carmona S, Simpkinson M, Lall D, Wu K, Daughrity L, Dickson DW, Harms MB, Petrucelli L, Lee EB, Lutz CM, Baloh RH
Neuron 2015 Dec 2;88(5):892-901
Neuron 2015 Dec 2;88(5):892-901
Disparate Mutations Confer Therapeutic Gain of Hsp104 Function.
Jackrel ME, Yee K, Tariq A, Chen AI, Shorter J
ACS chemical biology 2015 Dec 18;10(12):2672-9
ACS chemical biology 2015 Dec 18;10(12):2672-9
An age-related reduction of brain TBPH/TDP-43 levels precedes the onset of locomotion defects in a Drosophila ALS model.
Cragnaz L, Klima R, De Conti L, Romano G, Feiguin F, Buratti E, Baralle M, Baralle FE
Neuroscience 2015 Dec 17;311:415-21
Neuroscience 2015 Dec 17;311:415-21
Myofibrillar disruption and RNA-binding protein aggregation in a mouse model of limb-girdle muscular dystrophy 1D.
Bengoechea R, Pittman SK, Tuck EP, True HL, Weihl CC
Human molecular genetics 2015 Dec 1;24(23):6588-602
Human molecular genetics 2015 Dec 1;24(23):6588-602
R47H Variant of TREM2 Associated With Alzheimer Disease in a Large Late-Onset Family: Clinical, Genetic, and Neuropathological Study.
Korvatska O, Leverenz JB, Jayadev S, McMillan P, Kurtz I, Guo X, Rumbaugh M, Matsushita M, Girirajan S, Dorschner MO, Kiianitsa K, Yu CE, Brkanac Z, Garden GA, Raskind WH, Bird TD
JAMA neurology 2015 Aug;72(8):920-7
JAMA neurology 2015 Aug;72(8):920-7
G1/S Cell Cycle Checkpoint Dysfunction in Lymphoblasts from Sporadic Parkinson's Disease Patients.
Esteras N, Alquézar C, Bartolomé F, de la Encarnación A, Bermejo-Pareja F, Molina JA, Martín-Requero Á
Molecular neurobiology 2015 Aug;52(1):386-98
Molecular neurobiology 2015 Aug;52(1):386-98
Identification of transactivation-responsive DNA-binding protein 43 (TARDBP43; TDP-43) as a novel factor for TNF-α expression upon lipopolysaccharide stimulation in human monocytes.
Murata H, Hattori T, Maeda H, Takashiba S, Takigawa M, Kido J, Nagata T
Journal of periodontal research 2015 Aug;50(4):452-60
Journal of periodontal research 2015 Aug;50(4):452-60
Evaluation of Skin Fibroblasts from Amyotrophic Lateral Sclerosis Patients for the Rapid Study of Pathological Features.
Yang S, Zhang KY, Kariawasam R, Bax M, Fifita JA, Ooi L, Yerbury JJ, Nicholson GA, Blair IP
Neurotoxicity research 2015 Aug;28(2):138-46
Neurotoxicity research 2015 Aug;28(2):138-46
Expanding the spectrum of neuronal pathology in multiple system atrophy.
Cykowski MD, Coon EA, Powell SZ, Jenkins SM, Benarroch EE, Low PA, Schmeichel AM, Parisi JE
Brain : a journal of neurology 2015 Aug;138(Pt 8):2293-309
Brain : a journal of neurology 2015 Aug;138(Pt 8):2293-309
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD.
Ling JP, Pletnikova O, Troncoso JC, Wong PC
Science (New York, N.Y.) 2015 Aug 7;349(6248):650-5
Science (New York, N.Y.) 2015 Aug 7;349(6248):650-5
Traumatic brain injury causes frontotemporal dementia and TDP-43 proteolysis.
Wang HK, Lee YC, Huang CY, Liliang PC, Lu K, Chen HJ, Li YC, Tsai KJ
Neuroscience 2015 Aug 6;300:94-103
Neuroscience 2015 Aug 6;300:94-103
SQSTM1 splice site mutation in distal myopathy with rimmed vacuoles.
Bucelli RC, Arhzaouy K, Pestronk A, Pittman SK, Rojas L, Sue CM, Evilä A, Hackman P, Udd B, Harms MB, Weihl CC
Neurology 2015 Aug 25;85(8):665-74
Neurology 2015 Aug 25;85(8):665-74
Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions.
Farrawell NE, Lambert-Smith IA, Warraich ST, Blair IP, Saunders DN, Hatters DM, Yerbury JJ
Scientific reports 2015 Aug 21;5:13416
Scientific reports 2015 Aug 21;5:13416
Age-Dependent TDP-43-Mediated Motor Neuron Degeneration Requires GSK3, hat-trick, and xmas-2.
Sreedharan J, Neukomm LJ, Brown RH Jr, Freeman MR
Current biology : CB 2015 Aug 17;25(16):2130-6
Current biology : CB 2015 Aug 17;25(16):2130-6
A phosphomimetic mutant TDP-43 (S409/410E) induces Drosha instability and cytotoxicity in Neuro 2A cells.
Kim KY, Lee HW, Shim YM, Mook-Jung I, Jeon GS, Sung JJ
Biochemical and biophysical research communications 2015 Aug 14;464(1):236-43
Biochemical and biophysical research communications 2015 Aug 14;464(1):236-43
An optimized InCell Western screening technique identifies hexachlorophene as a novel potent TDP43 targeting drug.
Narayan M, Peralta DA, Gibson C, Zitnyar A, Jinwal UK
Journal of biotechnology 2015 Aug 10;207:34-8
Journal of biotechnology 2015 Aug 10;207:34-8
Initial gene vector dosing for studying symptomatology of amyotrophic lateral sclerosis in non-human primates.
Jackson KL, Dayton RD, Fisher-Perkins JM, Didier PJ, Baker KC, Weimer M, Gutierrez A, Cain CD, Mathis JM, Gitcho MA, Bunnell BA, Klein RL
Journal of medical primatology 2015 Apr;44(2):66-75
Journal of medical primatology 2015 Apr;44(2):66-75
Peptidylprolyl isomerase A governs TARDBP function and assembly in heterogeneous nuclear ribonucleoprotein complexes.
Lauranzano E, Pozzi S, Pasetto L, Stucchi R, Massignan T, Paolella K, Mombrini M, Nardo G, Lunetta C, Corbo M, Mora G, Bendotti C, Bonetto V
Brain : a journal of neurology 2015 Apr;138(Pt 4):974-91
Brain : a journal of neurology 2015 Apr;138(Pt 4):974-91
A novel mutation P112H in the TARDBP gene associated with frontotemporal lobar degeneration without motor neuron disease and abundant neuritic amyloid plaques.
Moreno F, Rabinovici GD, Karydas A, Miller Z, Hsu SC, Legati A, Fong J, Schonhaut D, Esselmann H, Watson C, Stephens ML, Kramer J, Wiltfang J, Seeley WW, Miller BL, Coppola G, Grinberg LT
Acta neuropathologica communications 2015 Apr 3;3:19
Acta neuropathologica communications 2015 Apr 3;3:19
FUS/TLS deficiency causes behavioral and pathological abnormalities distinct from amyotrophic lateral sclerosis.
Kino Y, Washizu C, Kurosawa M, Yamada M, Miyazaki H, Akagi T, Hashikawa T, Doi H, Takumi T, Hicks GG, Hattori N, Shimogori T, Nukina N
Acta neuropathologica communications 2015 Apr 25;3:24
Acta neuropathologica communications 2015 Apr 25;3:24
G3BP1 promotes stress-induced RNA granule interactions to preserve polyadenylated mRNA.
Aulas A, Caron G, Gkogkas CG, Mohamed NV, Destroismaisons L, Sonenberg N, Leclerc N, Parker JA, Vande Velde C
The Journal of cell biology 2015 Apr 13;209(1):73-84
The Journal of cell biology 2015 Apr 13;209(1):73-84
TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodies.
Goossens J, Vanmechelen E, Trojanowski JQ, Lee VM, Van Broeckhoven C, van der Zee J, Engelborghs S
Acta neuropathologica communications 2015 Apr 1;3:15
Acta neuropathologica communications 2015 Apr 1;3:15
Phenotype of transgenic mice carrying a very low copy number of the mutant human G93A superoxide dismutase-1 gene associated with amyotrophic lateral sclerosis.
Deitch JS, Alexander GM, Bensinger A, Yang S, Jiang JT, Heiman-Patterson TD
PloS one 2014;9(6):e99879
PloS one 2014;9(6):e99879
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
Perera ND, Sheean RK, Scott JW, Kemp BE, Horne MK, Turner BJ
PloS one 2014;9(4):e95549
PloS one 2014;9(4):e95549
Gene targeting of mouse Tardbp negatively affects Masp2 expression.
Dib S, Xiao S, Miletic D, Robertson J
PloS one 2014;9(4):e95373
PloS one 2014;9(4):e95373
Allele-specific knockdown of ALS-associated mutant TDP-43 in neural stem cells derived from induced pluripotent stem cells.
Nishimura AL, Shum C, Scotter EL, Abdelgany A, Sardone V, Wright J, Lee YB, Chen HJ, Bilican B, Carrasco M, Maniatis T, Chandran S, Rogelj B, Gallo JM, Shaw CE
PloS one 2014;9(3):e91269
PloS one 2014;9(3):e91269
Casein kinase II induced polymerization of soluble TDP-43 into filaments is inhibited by heat shock proteins.
Carlomagno Y, Zhang Y, Davis M, Lin WL, Cook C, Dunmore J, Tay W, Menkosky K, Cao X, Petrucelli L, Deture M
PloS one 2014;9(3):e90452
PloS one 2014;9(3):e90452
Mutant TDP-43 deregulates AMPK activation by PP2A in ALS models.
Perera ND, Sheean RK, Scott JW, Kemp BE, Horne MK, Turner BJ
PloS one 2014;9(3):e90449
PloS one 2014;9(3):e90449
Th1 response and systemic treg deficiency in inclusion body myositis.
Allenbach Y, Chaara W, Rosenzwajg M, Six A, Prevel N, Mingozzi F, Wanschitz J, Musset L, Charuel JL, Eymard B, Salomon B, Duyckaerts C, Maisonobe T, Dubourg O, Herson S, Klatzmann D, Benveniste O
PloS one 2014;9(3):e88788
PloS one 2014;9(3):e88788
Divergent phenotypes in mutant TDP-43 transgenic mice highlight potential confounds in TDP-43 transgenic modeling.
D'Alton S, Altshuler M, Cannon A, Dickson DW, Petrucelli L, Lewis J
PloS one 2014;9(1):e86513
PloS one 2014;9(1):e86513
Increased metal content in the TDP-43(A315T) transgenic mouse model of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Dang TN, Lim NK, Grubman A, Li QX, Volitakis I, White AR, Crouch PJ
Frontiers in aging neuroscience 2014;6:15
Frontiers in aging neuroscience 2014;6:15
The co-occurrence of Alzheimer's disease and Huntington's disease: a neuropathological study of 15 elderly Huntington's disease subjects.
Davis MY, Keene CD, Jayadev S, Bird T
Journal of Huntington's disease 2014;3(2):209-17
Journal of Huntington's disease 2014;3(2):209-17
Common pathobiochemical hallmarks of progranulin-associated frontotemporal lobar degeneration and neuronal ceroid lipofuscinosis.
Götzl JK, Mori K, Damme M, Fellerer K, Tahirovic S, Kleinberger G, Janssens J, van der Zee J, Lang CM, Kremmer E, Martin JJ, Engelborghs S, Kretzschmar HA, Arzberger T, Van Broeckhoven C, Haass C, Capell A
Acta neuropathologica 2014;127(6):845-60
Acta neuropathologica 2014;127(6):845-60
TDP-43 regulates β-adducin (Add2) transcript stability.
Costessi L, Porro F, Iaconcig A, Muro AF
RNA biology 2014;11(10):1280-90
RNA biology 2014;11(10):1280-90
Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury.
Yang Z, Lin F, Robertson CS, Wang KK
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism 2014 Sep;34(9):1444-52
Journal of cerebral blood flow and metabolism : official journal of the International Society of Cerebral Blood Flow and Metabolism 2014 Sep;34(9):1444-52
Atypical Alzheimer's disease in an elderly United States resident with amyotrophic lateral sclerosis and pathological tau in spinal motor neurons.
McCluskey LF, Geser F, Elman LB, Van Deerlin VM, Robinson JL, Lee VM, Trojanowski JQ
Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 Sep;15(5-6):466-72
Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 Sep;15(5-6):466-72
RNA metabolism in ALS: when normal processes become pathological.
Droppelmann CA, Campos-Melo D, Ishtiaq M, Volkening K, Strong MJ
Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 Sep;15(5-6):321-36
Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 Sep;15(5-6):321-36
Corticobasal degeneration initially developing motor versus non-motor symptoms: a comparative clinicopathological study.
Ikeda C, Yokota O, Nagao S, Ishizu H, Morisada Y, Terada S, Nakashima Y, Akiyama H, Uchitomi Y
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society 2014 Sep;14(3):152-64
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society 2014 Sep;14(3):152-64
TDP-43 toxicity proceeds via calcium dysregulation and necrosis in aging Caenorhabditis elegans motor neurons.
Aggad D, Vérièpe J, Tauffenberger A, Parker JA
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Sep 3;34(36):12093-103
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Sep 3;34(36):12093-103
Early retinal neurodegeneration and impaired Ran-mediated nuclear import of TDP-43 in progranulin-deficient FTLD.
Ward ME, Taubes A, Chen R, Miller BL, Sephton CF, Gelfand JM, Minami S, Boscardin J, Martens LH, Seeley WW, Yu G, Herz J, Filiano AJ, Arrant AE, Roberson ED, Kraft TW, Farese RV Jr, Green A, Gan L
The Journal of experimental medicine 2014 Sep 22;211(10):1937-45
The Journal of experimental medicine 2014 Sep 22;211(10):1937-45
Parkin-mediated reduction of nuclear and soluble TDP-43 reverses behavioral decline in symptomatic mice.
Wenqiang C, Lonskaya I, Hebron ML, Ibrahim Z, Olszewski RT, Neale JH, Moussa CE
Human molecular genetics 2014 Sep 15;23(18):4960-9
Human molecular genetics 2014 Sep 15;23(18):4960-9
Brain injection of α-synuclein induces multiple proteinopathies, gliosis, and a neuronal injury marker.
Sacino AN, Brooks M, McKinney AB, Thomas MA, Shaw G, Golde TE, Giasson BI
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Sep 10;34(37):12368-78
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Sep 10;34(37):12368-78
Potentiated Hsp104 variants suppress toxicity of diverse neurodegenerative disease-linked proteins.
Jackrel ME, Shorter J
Disease models & mechanisms 2014 Oct;7(10):1175-84
Disease models & mechanisms 2014 Oct;7(10):1175-84
Loss of nuclear TDP-43 in amyotrophic lateral sclerosis (ALS) causes altered expression of splicing machinery and widespread dysregulation of RNA splicing in motor neurones.
Highley JR, Kirby J, Jansweijer JA, Webb PS, Hewamadduma CA, Heath PR, Higginbottom A, Raman R, Ferraiuolo L, Cooper-Knock J, McDermott CJ, Wharton SB, Shaw PJ, Ince PG
Neuropathology and applied neurobiology 2014 Oct;40(6):670-85
Neuropathology and applied neurobiology 2014 Oct;40(6):670-85
Hippocampal sclerosis dementia with the C9ORF72 hexanucleotide repeat expansion.
Pletnikova O, Sloane KL, Renton AE, Traynor BJ, Crain BJ, Reid T, Zu T, Ranum LP, Troncoso JC, Rabins PV, Onyike CU
Neurobiology of aging 2014 Oct;35(10):2419.e17-21
Neurobiology of aging 2014 Oct;35(10):2419.e17-21
FTLD-ALS of TDP-43 type and SCA2 in a family with a full ataxin-2 polyglutamine expansion.
Bäumer D, East SZ, Tseu B, Zeman A, Hilton D, Talbot K, Ansorge O
Acta neuropathologica 2014 Oct;128(4):597-604
Acta neuropathologica 2014 Oct;128(4):597-604
RNA-processing protein TDP-43 regulates FOXO-dependent protein quality control in stress response.
Zhang T, Baldie G, Periz G, Wang J
PLoS genetics 2014 Oct;10(10):e1004693
PLoS genetics 2014 Oct;10(10):e1004693
TDP-43 suppresses CGG repeat-induced neurotoxicity through interactions with HnRNP A2/B1.
He F, Krans A, Freibaum BD, Taylor JP, Todd PK
Human molecular genetics 2014 Oct 1;23(19):5036-51
Human molecular genetics 2014 Oct 1;23(19):5036-51
Concomitant accumulation of α-synuclein and TDP-43 in a patient with corticobasal degeneration.
Yamashita S, Sakashita N, Yamashita T, Tawara N, Tasaki M, Kawakami K, Komohara Y, Fujiwara Y, Kamikawa M, Nakagawa T, Hirano T, Maeda Y, Hasegawa M, Takeya M, Ando Y
Journal of neurology 2014 Nov;261(11):2209-17
Journal of neurology 2014 Nov;261(11):2209-17
ALS-associated peripherin spliced transcripts form distinct protein inclusions that are neuroprotective against oxidative stress.
McLean JR, Smith GA, Rocha EM, Osborn TM, Dib S, Hayes MA, Beagan JA, Brown TB, Lawson TF, Hallett PJ, Robertson J, Isacson O
Experimental neurology 2014 Nov;261:217-29
Experimental neurology 2014 Nov;261:217-29
Intrathecal infusion of BMAA induces selective motor neuron damage and astrogliosis in the ventral horn of the spinal cord.
Yin HZ, Yu S, Hsu CI, Liu J, Acab A, Wu R, Tao A, Chiang BJ, Weiss JH
Experimental neurology 2014 Nov;261:1-9
Experimental neurology 2014 Nov;261:1-9
Neuropathological features of genetically confirmed DYT1 dystonia: investigating disease-specific inclusions.
Paudel R, Kiely A, Li A, Lashley T, Bandopadhyay R, Hardy J, Jinnah HA, Bhatia K, Houlden H, Holton JL
Acta neuropathologica communications 2014 Nov 18;2:159
Acta neuropathologica communications 2014 Nov 18;2:159
CGG repeats in RNA modulate expression of TDP-43 in mouse and fly models of fragile X tremor ataxia syndrome.
Galloway JN, Shaw C, Yu P, Parghi D, Poidevin M, Jin P, Nelson DL
Human molecular genetics 2014 Nov 15;23(22):5906-15
Human molecular genetics 2014 Nov 15;23(22):5906-15
A novel GRN mutation (GRN c.708+6_+9delTGAG) in frontotemporal lobar degeneration with TDP-43-positive inclusions: clinicopathologic report of 6 cases.
Bit-Ivan EN, Suh E, Shim HS, Weintraub S, Hyman BT, Arnold SE, McCarty-Wood E, Van Deerlin VM, Schneider JA, Trojanowski JQ, Frosch MP, Baker MC, Rademakers R, Mesulam M, Bigio EH
Journal of neuropathology and experimental neurology 2014 May;73(5):467-73
Journal of neuropathology and experimental neurology 2014 May;73(5):467-73
Tar DNA-binding protein-43 (TDP-43) regulates axon growth in vitro and in vivo.
Tripathi VB, Baskaran P, Shaw CE, Guthrie S
Neurobiology of disease 2014 May;65(100):25-34
Neurobiology of disease 2014 May;65(100):25-34
Mutations in the Matrin 3 gene cause familial amyotrophic lateral sclerosis.
Johnson JO, Pioro EP, Boehringer A, Chia R, Feit H, Renton AE, Pliner HA, Abramzon Y, Marangi G, Winborn BJ, Gibbs JR, Nalls MA, Morgan S, Shoai M, Hardy J, Pittman A, Orrell RW, Malaspina A, Sidle KC, Fratta P, Harms MB, Baloh RH, Pestronk A, Weihl CC, Rogaeva E, Zinman L, Drory VE, Borghero G, Mora G, Calvo A, Rothstein JD, ITALSGEN, Drepper C, Sendtner M, Singleton AB, Taylor JP, Cookson MR, Restagno G, Sabatelli M, Bowser R, Chiò A, Traynor BJ
Nature neuroscience 2014 May;17(5):664-666
Nature neuroscience 2014 May;17(5):664-666
PRKAR1B mutation associated with a new neurodegenerative disorder with unique pathology.
Wong TH, Chiu WZ, Breedveld GJ, Li KW, Verkerk AJ, Hondius D, Hukema RK, Seelaar H, Frick P, Severijnen LA, Lammers GJ, Lebbink JH, van Duinen SG, Kamphorst W, Rozemuller AJ, Netherlands Brain Bank, Bakker EB, International Parkinsonism Genetics Network, Neumann M, Willemsen R, Bonifati V, Smit AB, van Swieten J
Brain : a journal of neurology 2014 May;137(Pt 5):1361-73
Brain : a journal of neurology 2014 May;137(Pt 5):1361-73
Differential diagnosis of amyotrophic lateral sclerosis from Guillain-Barré syndrome by quantitative determination of TDP-43 in cerebrospinal fluid.
Hosokawa M, Arai T, Yamashita M, Tsuji H, Nonaka T, Masuda-Suzukake M, Tamaoka A, Hasegawa M, Akiyama H
The International journal of neuroscience 2014 May;124(5):344-9
The International journal of neuroscience 2014 May;124(5):344-9
Astrocytic TDP-43 pathology in Alexander disease.
Walker AK, Daniels CM, Goldman JE, Trojanowski JQ, Lee VM, Messing A
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 May 7;34(19):6448-58
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 May 7;34(19):6448-58
The RNA-binding protein TDP-43 selectively disrupts microRNA-1/206 incorporation into the RNA-induced silencing complex.
King IN, Yartseva V, Salas D, Kumar A, Heidersbach A, Ando DM, Stallings NR, Elliott JL, Srivastava D, Ivey KN
The Journal of biological chemistry 2014 May 16;289(20):14263-71
The Journal of biological chemistry 2014 May 16;289(20):14263-71
Differential motor neuron involvement in progressive muscular atrophy: a comparative study with amyotrophic lateral sclerosis.
Riku Y, Atsuta N, Yoshida M, Tatsumi S, Iwasaki Y, Mimuro M, Watanabe H, Ito M, Senda J, Nakamura R, Koike H, Sobue G
BMJ open 2014 May 14;4(5):e005213
BMJ open 2014 May 14;4(5):e005213
Hereditary myopathy with early respiratory failure: occurrence in various populations.
Palmio J, Evilä A, Chapon F, Tasca G, Xiang F, Brådvik B, Eymard B, Echaniz-Laguna A, Laporte J, Kärppä M, Mahjneh I, Quinlivan R, Laforêt P, Damian M, Berardo A, Taratuto AL, Bueri JA, Tommiska J, Raivio T, Tuerk M, Gölitz P, Chevessier F, Sewry C, Norwood F, Hedberg C, Schröder R, Edström L, Oldfors A, Hackman P, Udd B
Journal of neurology, neurosurgery, and psychiatry 2014 Mar;85(3):345-53
Journal of neurology, neurosurgery, and psychiatry 2014 Mar;85(3):345-53
Autopsy case of concurrent Huntington's disease and neurofibromatosis type 1.
Kawakami I, Katsuse O, Aoki N, Togo T, Suzuki K, Isojima D, Kondo D, Iseki E, Kosaka K, Akiyama H, Hirayasu Y
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society 2014 Mar;14(1):81-6
Psychogeriatrics : the official journal of the Japanese Psychogeriatric Society 2014 Mar;14(1):81-6
Early dipeptide repeat pathology in a frontotemporal dementia kindred with C9ORF72 mutation and intellectual disability.
Proudfoot M, Gutowski NJ, Edbauer D, Hilton DA, Stephens M, Rankin J, Mackenzie IR
Acta neuropathologica 2014 Mar;127(3):451-8
Acta neuropathologica 2014 Mar;127(3):451-8
Staging TDP-43 pathology in Alzheimer's disease.
Josephs KA, Murray ME, Whitwell JL, Parisi JE, Petrucelli L, Jack CR, Petersen RC, Dickson DW
Acta neuropathologica 2014 Mar;127(3):441-50
Acta neuropathologica 2014 Mar;127(3):441-50
ALS-linked mutations enlarge TDP-43-enriched neuronal RNA granules in the dendritic arbor.
Liu-Yesucevitz L, Lin AY, Ebata A, Boon JY, Reid W, Xu YF, Kobrin K, Murphy GJ, Petrucelli L, Wolozin B
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Mar 19;34(12):4167-74
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Mar 19;34(12):4167-74
Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species.
Scotter EL, Vance C, Nishimura AL, Lee YB, Chen HJ, Urwin H, Sardone V, Mitchell JC, Rogelj B, Rubinsztein DC, Shaw CE
Journal of cell science 2014 Mar 15;127(Pt 6):1263-78
Journal of cell science 2014 Mar 15;127(Pt 6):1263-78
Plasma phosphorylated TDP-43 levels are elevated in patients with frontotemporal dementia carrying a C9orf72 repeat expansion or a GRN mutation.
Suárez-Calvet M, Dols-Icardo O, Lladó A, Sánchez-Valle R, Hernández I, Amer G, Antón-Aguirre S, Alcolea D, Fortea J, Ferrer I, van der Zee J, Dillen L, Van Broeckhoven C, Molinuevo JL, Blesa R, Clarimón J, Lleó A
Journal of neurology, neurosurgery, and psychiatry 2014 Jun;85(6):684-91
Journal of neurology, neurosurgery, and psychiatry 2014 Jun;85(6):684-91
Neuropathological features of multiple system atrophy with cognitive impairment.
Asi YT, Ling H, Ahmed Z, Lees AJ, Revesz T, Holton JL
Movement disorders : official journal of the Movement Disorder Society 2014 Jun;29(7):884-8
Movement disorders : official journal of the Movement Disorder Society 2014 Jun;29(7):884-8
Argyrophilic grain disease as a neurodegenerative substrate in late-onset schizophrenia and delusional disorders.
Nagao S, Yokota O, Ikeda C, Takeda N, Ishizu H, Kuroda S, Sudo K, Terada S, Murayama S, Uchitomi Y
European archives of psychiatry and clinical neuroscience 2014 Jun;264(4):317-31
European archives of psychiatry and clinical neuroscience 2014 Jun;264(4):317-31
TAR DNA-binding protein 43 pathology in Alzheimer's disease with psychosis.
Vatsavayi AV, Kofler J, Demichele-Sweet MA, Murray PS, Lopez OL, Sweet RA
International psychogeriatrics 2014 Jun;26(6):987-94
International psychogeriatrics 2014 Jun;26(6):987-94
Frontotemporal lobar degeneration with writing disturbance mainly consisting of omission of kana letters.
Shimizu T, Mochizuki Y, Bandoh M, Taguchi T, Mishima K, Takeda K, Nojima K, Mizutani T, Matsubara S
Neurocase 2014 Jun;20(3):355-60
Neurocase 2014 Jun;20(3):355-60
FUS is phosphorylated by DNA-PK and accumulates in the cytoplasm after DNA damage.
Deng Q, Holler CJ, Taylor G, Hudson KF, Watkins W, Gearing M, Ito D, Murray ME, Dickson DW, Seyfried NT, Kukar T
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Jun 4;34(23):7802-13
The Journal of neuroscience : the official journal of the Society for Neuroscience 2014 Jun 4;34(23):7802-13
Mitochondrial dysfunction and decrease in body weight of transgenic knock-in mouse model for TDP-43: the question of glucose?
Jawaid A, Gapp K, Schulz PE
The Journal of biological chemistry 2014 Jun 27;289(26):18593
The Journal of biological chemistry 2014 Jun 27;289(26):18593
Brain distribution of dipeptide repeat proteins in frontotemporal lobar degeneration and motor neurone disease associated with expansions in C9ORF72.
Davidson YS, Barker H, Robinson AC, Thompson JC, Harris J, Troakes C, Smith B, Al-Saraj S, Shaw C, Rollinson S, Masuda-Suzukake M, Hasegawa M, Pickering-Brown S, Snowden JS, Mann DM
Acta neuropathologica communications 2014 Jun 20;2:70
Acta neuropathologica communications 2014 Jun 20;2:70
Partial deletion of AFG3L2 causing spinocerebellar ataxia type 28.
Smets K, Deconinck T, Baets J, Sieben A, Martin JJ, Smouts I, Wang S, Taroni F, Di Bella D, Van Hecke W, Parizel PM, Jadoul C, De Potter R, Couvreur F, Rugarli E, De Jonghe P
Neurology 2014 Jun 10;82(23):2092-100
Neurology 2014 Jun 10;82(23):2092-100
Abnormal distribution of heterogeneous nuclear ribonucleoproteins in sporadic inclusion body myositis.
Pinkus JL, Amato AA, Taylor JP, Greenberg SA
Neuromuscular disorders : NMD 2014 Jul;24(7):611-6
Neuromuscular disorders : NMD 2014 Jul;24(7):611-6
Neuropathologic assessment of dementia markers in identical and fraternal twins.
Iacono D, Volkman I, Nennesmo I, Pedersen NL, Fratiglioni L, Johansson B, Karlsson D, Winblad B, Gatz M
Brain pathology (Zurich, Switzerland) 2014 Jul;24(4):317-33
Brain pathology (Zurich, Switzerland) 2014 Jul;24(4):317-33
Sequestration of multiple RNA recognition motif-containing proteins by C9orf72 repeat expansions.
Cooper-Knock J, Walsh MJ, Higginbottom A, Robin Highley J, Dickman MJ, Edbauer D, Ince PG, Wharton SB, Wilson SA, Kirby J, Hautbergue GM, Shaw PJ
Brain : a journal of neurology 2014 Jul;137(Pt 7):2040-51
Brain : a journal of neurology 2014 Jul;137(Pt 7):2040-51
UBE2E ubiquitin-conjugating enzymes and ubiquitin isopeptidase Y regulate TDP-43 protein ubiquitination.
Hans F, Fiesel FC, Strong JC, Jäckel S, Rasse TM, Geisler S, Springer W, Schulz JB, Voigt A, Kahle PJ
The Journal of biological chemistry 2014 Jul 4;289(27):19164-79
The Journal of biological chemistry 2014 Jul 4;289(27):19164-79
Myopathy-causing mutations in an HSP40 chaperone disrupt processing of specific client conformers.
Stein KC, Bengoechea R, Harms MB, Weihl CC, True HL
The Journal of biological chemistry 2014 Jul 25;289(30):21120-30
The Journal of biological chemistry 2014 Jul 25;289(30):21120-30
RPL24: a potential therapeutic target whose depletion or acetylation inhibits polysome assembly and cancer cell growth.
Wilson-Edell KA, Kehasse A, Scott GK, Yau C, Rothschild DE, Schilling B, Gabriel BS, Yevtushenko MA, Hanson IM, Held JM, Gibson BW, Benz CC
Oncotarget 2014 Jul 15;5(13):5165-76
Oncotarget 2014 Jul 15;5(13):5165-76
Possible involvement of lysosomal dysfunction in pathological changes of the brain in aged progranulin-deficient mice.
Tanaka Y, Chambers JK, Matsuwaki T, Yamanouchi K, Nishihara M
Acta neuropathologica communications 2014 Jul 15;2:78
Acta neuropathologica communications 2014 Jul 15;2:78
Metabolism and mis-metabolism of the neuropathological signature protein TDP-43.
Huang CC, Bose JK, Majumder P, Lee KH, Huang JT, Huang JK, Shen CK
Journal of cell science 2014 Jul 15;127(Pt 14):3024-38
Journal of cell science 2014 Jul 15;127(Pt 14):3024-38
Titinopathy in a Canadian family sharing the British founder haplotype.
Pfeffer G, Joseph JT, Innes AM, Frizzell JB, Wilson IJ, Brownell AK, Chinnery PF
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2014 Jan;41(1):90-4
The Canadian journal of neurological sciences. Le journal canadien des sciences neurologiques 2014 Jan;41(1):90-4
Accumulation of C-terminal fragments of transactive response DNA-binding protein 43 leads to synaptic loss and cognitive deficits in human TDP-43 transgenic mice.
Medina DX, Orr ME, Oddo S
Neurobiology of aging 2014 Jan;35(1):79-87
Neurobiology of aging 2014 Jan;35(1):79-87
A high-content screen identifies novel compounds that inhibit stress-induced TDP-43 cellular aggregation and associated cytotoxicity.
Boyd JD, Lee P, Feiler MS, Zauur N, Liu M, Concannon J, Ebata A, Wolozin B, Glicksman MA
Journal of biomolecular screening 2014 Jan;19(1):44-56
Journal of biomolecular screening 2014 Jan;19(1):44-56
Dendritic retraction, but not atrophy, is consistent in amyotrophic lateral sclerosis-comparison between Onuf's neurons and other sacral motor neurons.
Takeda T, Uchihara T, Nakayama Y, Nakamura A, Sasaki S, Kakei S, Uchiyama S, Duyckaerts C, Yoshida M
Acta neuropathologica communications 2014 Jan 27;2:11
Acta neuropathologica communications 2014 Jan 27;2:11
Potentiated Hsp104 variants antagonize diverse proteotoxic misfolding events.
Jackrel ME, DeSantis ME, Martinez BA, Castellano LM, Stewart RM, Caldwell KA, Caldwell GA, Shorter J
Cell 2014 Jan 16;156(1-2):170-82
Cell 2014 Jan 16;156(1-2):170-82
Prion-like nuclear aggregation of TDP-43 during heat shock is regulated by HSP40/70 chaperones.
Udan-Johns M, Bengoechea R, Bell S, Shao J, Diamond MI, True HL, Weihl CC, Baloh RH
Human molecular genetics 2014 Jan 1;23(1):157-70
Human molecular genetics 2014 Jan 1;23(1):157-70
De novo prion aggregates trigger autophagy in skeletal muscle.
Joshi-Barr S, Bett C, Chiang WC, Trejo M, Goebel HH, Sikorska B, Liberski P, Raeber A, Lin JH, Masliah E, Sigurdson CJ
Journal of virology 2014 Feb;88(4):2071-82
Journal of virology 2014 Feb;88(4):2071-82
Lower motor neuron involvement in TAR DNA-binding protein of 43 kDa-related frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Riku Y, Watanabe H, Yoshida M, Tatsumi S, Mimuro M, Iwasaki Y, Katsuno M, Iguchi Y, Masuda M, Senda J, Ishigaki S, Udagawa T, Sobue G
JAMA neurology 2014 Feb;71(2):172-9
JAMA neurology 2014 Feb;71(2):172-9
Therapeutic modulation of eIF2α phosphorylation rescues TDP-43 toxicity in amyotrophic lateral sclerosis disease models.
Kim HJ, Raphael AR, LaDow ES, McGurk L, Weber RA, Trojanowski JQ, Lee VM, Finkbeiner S, Gitler AD, Bonini NM
Nature genetics 2014 Feb;46(2):152-60
Nature genetics 2014 Feb;46(2):152-60
Clinicopathologic features of autosomal recessive amyotrophic lateral sclerosis associated with optineurin mutation.
Kamada M, Izumi Y, Ayaki T, Nakamura M, Kagawa S, Kudo E, Sako W, Maruyama H, Nishida Y, Kawakami H, Ito H, Kaji R
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Feb;34(1):64-70
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Feb;34(1):64-70
NF-Y inactivation causes atypical neurodegeneration characterized by ubiquitin and p62 accumulation and endoplasmic reticulum disorganization.
Yamanaka T, Tosaki A, Kurosawa M, Matsumoto G, Koike M, Uchiyama Y, Maity SN, Shimogori T, Hattori N, Nukina N
Nature communications 2014 Feb 25;5:3354
Nature communications 2014 Feb 25;5:3354
TDP-43 Phosphorylation by casein kinase Iε promotes oligomerization and enhances toxicity in vivo.
Choksi DK, Roy B, Chatterjee S, Yusuff T, Bakhoum MF, Sengupta U, Ambegaokar S, Kayed R, Jackson GR
Human molecular genetics 2014 Feb 15;23(4):1025-35
Human molecular genetics 2014 Feb 15;23(4):1025-35
No interaction between tau and TDP-43 pathologies in either frontotemporal lobar degeneration or motor neurone disease.
Robinson AC, Thompson JC, Weedon L, Rollinson S, Pickering-Brown S, Snowden JS, Davidson YS, Mann DM
Neuropathology and applied neurobiology 2014 Dec;40(7):844-54
Neuropathology and applied neurobiology 2014 Dec;40(7):844-54
Cytoplasmic TDP-43 accumulation in cells of the adrenal medulla in individuals with or without amyotrophic lateral sclerosis.
Okamoto K, Amari M, Fujita Y, Makioka K, Fukuda T, Suzuki K, Takatama M
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Dec;34(6):535-40
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Dec;34(6):535-40
H63D HFE genotype accelerates disease progression in animal models of amyotrophic lateral sclerosis.
Nandar W, Neely EB, Simmons Z, Connor JR
Biochimica et biophysica acta 2014 Dec;1842(12 Pt A):2413-26
Biochimica et biophysica acta 2014 Dec;1842(12 Pt A):2413-26
TDP-43 pathology in the basal forebrain and hypothalamus of patients with amyotrophic lateral sclerosis.
Cykowski MD, Takei H, Schulz PE, Appel SH, Powell SZ
Acta neuropathologica communications 2014 Dec 24;2:171
Acta neuropathologica communications 2014 Dec 24;2:171
An ALS-mutant TDP-43 neurotoxic peptide adopts an anti-parallel β-structure and induces TDP-43 redistribution.
Zhu L, Xu M, Yang M, Yang Y, Li Y, Deng J, Ruan L, Liu J, Du S, Liu X, Feng W, Fushimi K, Bigio EH, Mesulam M, Wang C, Wu JY
Human molecular genetics 2014 Dec 20;23(25):6863-77
Human molecular genetics 2014 Dec 20;23(25):6863-77
Aggregation properties of the small nuclear ribonucleoprotein U1-70K in Alzheimer disease.
Diner I, Hales CM, Bishof I, Rabenold L, Duong DM, Yi H, Laur O, Gearing M, Troncoso J, Thambisetty M, Lah JJ, Levey AI, Seyfried NT
The Journal of biological chemistry 2014 Dec 19;289(51):35296-313
The Journal of biological chemistry 2014 Dec 19;289(51):35296-313
Proteasome dysfunction induces muscle growth defects and protein aggregation.
Kitajima Y, Tashiro Y, Suzuki N, Warita H, Kato M, Tateyama M, Ando R, Izumi R, Yamazaki M, Abe M, Sakimura K, Ito H, Urushitani M, Nagatomi R, Takahashi R, Aoki M
Journal of cell science 2014 Dec 15;127(Pt 24):5204-17
Journal of cell science 2014 Dec 15;127(Pt 24):5204-17
Immunoreactivity of valosin-containing protein in sporadic amyotrophic lateral sclerosis and in a case of its novel mutant.
Ayaki T, Ito H, Fukushima H, Inoue T, Kondo T, Ikemoto A, Asano T, Shodai A, Fujita T, Fukui S, Morino H, Nakano S, Kusaka H, Yamashita H, Ihara M, Matsumoto R, Kawamata J, Urushitani M, Kawakami H, Takahashi R
Acta neuropathologica communications 2014 Dec 10;2:172
Acta neuropathologica communications 2014 Dec 10;2:172
New criteria for frontotemporal dementia syndromes: clinical and pathological diagnostic implications.
Chare L, Hodges JR, Leyton CE, McGinley C, Tan RH, Kril JJ, Halliday GM
Journal of neurology, neurosurgery, and psychiatry 2014 Aug;85(8):865-70
Journal of neurology, neurosurgery, and psychiatry 2014 Aug;85(8):865-70
The voltage-gated calcium channel blocker lomerizine is neuroprotective in motor neurons expressing mutant SOD1, but not TDP-43.
Tran LT, Gentil BJ, Sullivan KE, Durham HD
Journal of neurochemistry 2014 Aug;130(3):455-66
Journal of neurochemistry 2014 Aug;130(3):455-66
Autophagy induction enhances TDP43 turnover and survival in neuronal ALS models.
Barmada SJ, Serio A, Arjun A, Bilican B, Daub A, Ando DM, Tsvetkov A, Pleiss M, Li X, Peisach D, Shaw C, Chandran S, Finkbeiner S
Nature chemical biology 2014 Aug;10(8):677-85
Nature chemical biology 2014 Aug;10(8):677-85
FUS colocalizes with polyglutamine, but not with TDP-43 in neuronal intranuclear inclusions in spinocerebellar ataxia type 2.
Mori F, Toyoshima Y, Tanji K, Kakita A, Takahashi H, Wakabayashi K
Neuropathology and applied neurobiology 2014 Apr;40(3):351-5
Neuropathology and applied neurobiology 2014 Apr;40(3):351-5
Accumulation of the sigma-1 receptor is common to neuronal nuclear inclusions in various neurodegenerative diseases.
Miki Y, Mori F, Kon T, Tanji K, Toyoshima Y, Yoshida M, Sasaki H, Kakita A, Takahashi H, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Apr;34(2):148-58
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Apr;34(2):148-58
Ubiquitin-negative, eosinophilic neuronal cytoplasmic inclusions associated with stress granules and autophagy: an immunohistochemical investigation of two cases.
Mori F, Watanabe Y, Miki Y, Tanji K, Odagiri S, Eto K, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Apr;34(2):140-7
Neuropathology : official journal of the Japanese Society of Neuropathology 2014 Apr;34(2):140-7
Reduced cellular Ca(2+) availability enhances TDP-43 cleavage by apoptotic caspases.
De Marco G, Lomartire A, Mandili G, Lupino E, Buccinnà B, Ramondetti C, Moglia C, Novelli F, Piccinini M, Mostert M, Rinaudo MT, Chiò A, Calvo A
Biochimica et biophysica acta 2014 Apr;1843(4):725-34
Biochimica et biophysica acta 2014 Apr;1843(4):725-34
Parkin reverses TDP-43-induced cell death and failure of amino acid homeostasis.
Hebron M, Chen W, Miessau MJ, Lonskaya I, Moussa CE
Journal of neurochemistry 2014 Apr;129(2):350-61
Journal of neurochemistry 2014 Apr;129(2):350-61
A retrospective cohort study identifying the principal pathological features useful in the diagnosis of inclusion body myositis.
Brady S, Squier W, Sewry C, Hanna M, Hilton-Jones D, Holton JL
BMJ open 2014 Apr 28;4(4):e004552
BMJ open 2014 Apr 28;4(4):e004552
A novel approach for integrative studies on neurodegenerative diseases in human brains.
Theofilas P, Polichiso L, Wang X, Lima LC, Alho ATL, Leite REP, Suemoto CK, Pasqualucci CA, Jacob-Filho W, Heinsen H, Brazilian Aging Brain Study Group, Grinberg LT
Journal of neuroscience methods 2014 Apr 15;226:171-183
Journal of neuroscience methods 2014 Apr 15;226:171-183
Mitochondrial dysfunction and decrease in body weight of a transgenic knock-in mouse model for TDP-43.
Stribl C, Samara A, Trümbach D, Peis R, Neumann M, Fuchs H, Gailus-Durner V, Hrabě de Angelis M, Rathkolb B, Wolf E, Beckers J, Horsch M, Neff F, Kremmer E, Koob S, Reichert AS, Hans W, Rozman J, Klingenspor M, Aichler M, Walch AK, Becker L, Klopstock T, Glasl L, Hölter SM, Wurst W, Floss T
The Journal of biological chemistry 2014 Apr 11;289(15):10769-10784
The Journal of biological chemistry 2014 Apr 11;289(15):10769-10784
Diagnosis and management of behavioral variant frontotemporal dementia.
Pressman PS, Miller BL
Biological psychiatry 2014 Apr 1;75(7):574-81
Biological psychiatry 2014 Apr 1;75(7):574-81
TDP-43, an ALS linked protein, regulates fat deposition and glucose homeostasis.
Stallings NR, Puttaparthi K, Dowling KJ, Luther CM, Burns DK, Davis K, Elliott JL
PloS one 2013;8(8):e71793
PloS one 2013;8(8):e71793
Kinase Inhibitor Screening Identifies Cyclin-Dependent Kinases and Glycogen Synthase Kinase 3 as Potential Modulators of TDP-43 Cytosolic Accumulation during Cell Stress.
Moujalled D, James JL, Parker SJ, Lidgerwood GE, Duncan C, Meyerowitz J, Nonaka T, Hasegawa M, Kanninen KM, Grubman A, Liddell JR, Crouch PJ, White AR
PloS one 2013;8(6):e67433
PloS one 2013;8(6):e67433
RNP2 of RNA recognition motif 1 plays a central role in the aberrant modification of TDP-43.
Takagi S, Iguchi Y, Katsuno M, Ishigaki S, Ikenaka K, Fujioka Y, Honda D, Niwa J, Tanaka F, Watanabe H, Adachi H, Sobue G
PloS one 2013;8(6):e66966
PloS one 2013;8(6):e66966
A 43-kDa TDP-43 species is present in aggregates associated with frontotemporal lobar degeneration.
Bosque PJ, Boyer PJ, Mishra P
PloS one 2013;8(5):e62301
PloS one 2013;8(5):e62301
Characterization of the distal polyadenylation site of the ß-adducin (Add2) pre-mRNA.
Costessi L, Porro F, Iaconcig A, Nedeljkovic M, Muro AF
PloS one 2013;8(3):e58879
PloS one 2013;8(3):e58879
Identification of genetic modifiers of TDP-43 neurotoxicity in Drosophila.
Zhan L, Hanson KA, Kim SH, Tare A, Tibbetts RS
PloS one 2013;8(2):e57214
PloS one 2013;8(2):e57214
ALS-associated TDP-43 induces endoplasmic reticulum stress, which drives cytoplasmic TDP-43 accumulation and stress granule formation.
Walker AK, Soo KY, Sundaramoorthy V, Parakh S, Ma Y, Farg MA, Wallace RH, Crouch PJ, Turner BJ, Horne MK, Atkin JD
PloS one 2013;8(11):e81170
PloS one 2013;8(11):e81170
Downregulation of microRNA-9 in iPSC-derived neurons of FTD/ALS patients with TDP-43 mutations.
Zhang Z, Almeida S, Lu Y, Nishimura AL, Peng L, Sun D, Wu B, Karydas AM, Tartaglia MC, Fong JC, Miller BL, Farese RV Jr, Moore MJ, Shaw CE, Gao FB
PloS one 2013;8(10):e76055
PloS one 2013;8(10):e76055
Prevalence of dementia subtypes in a developing country: a clinicopathological study.
Grinberg LT, Nitrini R, Suemoto CK, Lucena Ferretti-Rebustini RE, Leite RE, Farfel JM, Santos E, Andrade MP, Alho AT, Lima Mdo C, Oliveira KC, Tampellini E, Polichiso L, Santos GB, Rodriguez RD, Ueda K, Pasqualucci CA, Jacob-Filho W
Clinics (Sao Paulo, Brazil) 2013;68(8):1140-5
Clinics (Sao Paulo, Brazil) 2013;68(8):1140-5
The ALS/FTLD-related RNA-binding proteins TDP-43 and FUS have common downstream RNA targets in cortical neurons.
Honda D, Ishigaki S, Iguchi Y, Fujioka Y, Udagawa T, Masuda A, Ohno K, Katsuno M, Sobue G
FEBS open bio 2013;4:1-10
FEBS open bio 2013;4:1-10
β-N-methylamino-L-alanine induces changes in both GSK3 and TDP-43 in human neuroblastoma.
Muñoz-Saez E, de Munck E, Arahuetes RM, Solas MT, Martínez AM, Miguel BG
The Journal of toxicological sciences 2013;38(3):425-30
The Journal of toxicological sciences 2013;38(3):425-30
Clinicopathologic study of Alzheimer's disease: Alzheimer mimics.
Shim YS, Roe CM, Buckles VD, Morris JC
Journal of Alzheimer's disease : JAD 2013;35(4):799-811
Journal of Alzheimer's disease : JAD 2013;35(4):799-811
ApoE and TDP-43 neuropathology in two siblings with familial FTLD-motor neuron disease.
Vossel KA, Bien-Ly N, Bernardo A, Rascovsky K, Karydas A, Rabinovici GD, Sidhu M, Huang EJ, Miller BL, Huang Y, Seeley WW
Neurocase 2013;19(3):295-301
Neurocase 2013;19(3):295-301
Neuropathology of partial PGC-1α deficiency recapitulates features of mitochondrial encephalopathies but not of neurodegenerative diseases.
Szalardy L, Zadori D, Plangar I, Vecsei L, Weydt P, Ludolph AC, Klivenyi P, Kovacs GG
Neuro-degenerative diseases 2013;12(4):177-88
Neuro-degenerative diseases 2013;12(4):177-88
Activation of transforming growth factor-β/Smad signaling reduces aggregate formation of mislocalized TAR DNA-binding protein-43.
Nakamura M, Kaneko S, Ito H, Jiang S, Fujita K, Wate R, Nakano S, Fujisawa J, Kusaka H
Neuro-degenerative diseases 2013;11(4):182-93
Neuro-degenerative diseases 2013;11(4):182-93
A cellular model for sporadic ALS using patient-derived induced pluripotent stem cells.
Burkhardt MF, Martinez FJ, Wright S, Ramos C, Volfson D, Mason M, Garnes J, Dang V, Lievers J, Shoukat-Mumtaz U, Martinez R, Gai H, Blake R, Vaisberg E, Grskovic M, Johnson C, Irion S, Bright J, Cooper B, Nguyen L, Griswold-Prenner I, Javaherian A
Molecular and cellular neurosciences 2013 Sep;56:355-64
Molecular and cellular neurosciences 2013 Sep;56:355-64
β-N-methylamino-l-alanine causes neurological and pathological phenotypes mimicking Amyotrophic Lateral Sclerosis (ALS): the first step towards an experimental model for sporadic ALS.
de Munck E, Muñoz-Sáez E, Miguel BG, Solas MT, Ojeda I, Martínez A, Gil C, Arahuetes RM
Environmental toxicology and pharmacology 2013 Sep;36(2):243-255
Environmental toxicology and pharmacology 2013 Sep;36(2):243-255
Early and selective reduction of NOP56 (Asidan) and RNA processing proteins in the motor neuron of ALS model mice.
Miyazaki K, Yamashita T, Morimoto N, Sato K, Mimoto T, Kurata T, Ikeda Y, Abe K
Neurological research 2013 Sep;35(7):744-54
Neurological research 2013 Sep;35(7):744-54
Mutation analysis and immunopathological studies of PFN1 in familial and sporadic amyotrophic lateral sclerosis.
Yang S, Fifita JA, Williams KL, Warraich ST, Pamphlett R, Nicholson GA, Blair IP
Neurobiology of aging 2013 Sep;34(9):2235.e7-10
Neurobiology of aging 2013 Sep;34(9):2235.e7-10
Familial dementia with frontotemporal features associated with M146V presenilin-1 mutation.
Riudavets MA, Bartoloni L, Troncoso JC, Pletnikova O, St George-Hyslop P, Schultz M, Sevlever G, Allegri RF
Brain pathology (Zurich, Switzerland) 2013 Sep;23(5):595-600
Brain pathology (Zurich, Switzerland) 2013 Sep;23(5):595-600
Sporadic ALS with compound heterozygous mutations in the SQSTM1 gene.
Shimizu H, Toyoshima Y, Shiga A, Yokoseki A, Arakawa K, Sekine Y, Shimohata T, Ikeuchi T, Nishizawa M, Kakita A, Onodera O, Takahashi H
Acta neuropathologica 2013 Sep;126(3):453-9
Acta neuropathologica 2013 Sep;126(3):453-9
Modeling key pathological features of frontotemporal dementia with C9ORF72 repeat expansion in iPSC-derived human neurons.
Almeida S, Gascon E, Tran H, Chou HJ, Gendron TF, Degroot S, Tapper AR, Sellier C, Charlet-Berguerand N, Karydas A, Seeley WW, Boxer AL, Petrucelli L, Miller BL, Gao FB
Acta neuropathologica 2013 Sep;126(3):385-99
Acta neuropathologica 2013 Sep;126(3):385-99
Non-targeted identification of prions and amyloid-forming proteins from yeast and mammalian cells.
Kryndushkin D, Pripuzova N, Burnett BG, Shewmaker F
The Journal of biological chemistry 2013 Sep 20;288(38):27100-27111
The Journal of biological chemistry 2013 Sep 20;288(38):27100-27111
Frontotemporal lobar degeneration: diversity of FTLD lesions.
Seilhean D, Bielle F, Plu I, Duyckaerts C
Revue neurologique 2013 Oct;169(10):786-92
Revue neurologique 2013 Oct;169(10):786-92
Targeting RNA foci in iPSC-derived motor neurons from ALS patients with a C9ORF72 repeat expansion.
Sareen D, O'Rourke JG, Meera P, Muhammad AK, Grant S, Simpkinson M, Bell S, Carmona S, Ornelas L, Sahabian A, Gendron T, Petrucelli L, Baughn M, Ravits J, Harms MB, Rigo F, Bennett CF, Otis TS, Svendsen CN, Baloh RH
Science translational medicine 2013 Oct 23;5(208):208ra149
Science translational medicine 2013 Oct 23;5(208):208ra149
RNA toxicity from the ALS/FTD C9ORF72 expansion is mitigated by antisense intervention.
Donnelly CJ, Zhang PW, Pham JT, Haeusler AR, Mistry NA, Vidensky S, Daley EL, Poth EM, Hoover B, Fines DM, Maragakis N, Tienari PJ, Petrucelli L, Traynor BJ, Wang J, Rigo F, Bennett CF, Blackshaw S, Sattler R, Rothstein JD
Neuron 2013 Oct 16;80(2):415-28
Neuron 2013 Oct 16;80(2):415-28
Decreased number of Gemini of coiled bodies and U12 snRNA level in amyotrophic lateral sclerosis.
Ishihara T, Ariizumi Y, Shiga A, Kato T, Tan CF, Sato T, Miki Y, Yokoo M, Fujino T, Koyama A, Yokoseki A, Nishizawa M, Kakita A, Takahashi H, Onodera O
Human molecular genetics 2013 Oct 15;22(20):4136-47
Human molecular genetics 2013 Oct 15;22(20):4136-47
Rescue of amyotrophic lateral sclerosis phenotype in a mouse model by intravenous AAV9-ADAR2 delivery to motor neurons.
Yamashita T, Chai HL, Teramoto S, Tsuji S, Shimazaki K, Muramatsu S, Kwak S
EMBO molecular medicine 2013 Nov;5(11):1710-9
EMBO molecular medicine 2013 Nov;5(11):1710-9
Amyotrophic lateral sclerosis: an update on recent genetic insights.
Iguchi Y, Katsuno M, Ikenaka K, Ishigaki S, Sobue G
Journal of neurology 2013 Nov;260(11):2917-27
Journal of neurology 2013 Nov;260(11):2917-27
Quantitative neurofibrillary tangle density and brain volumetric MRI analyses in Alzheimer's disease presenting as logopenic progressive aphasia.
Josephs KA, Dickson DW, Murray ME, Senjem ML, Parisi JE, Petersen RC, Jack CR Jr, Whitwell JL
Brain and language 2013 Nov;127(2):127-34
Brain and language 2013 Nov;127(2):127-34
Targeted degradation of sense and antisense C9orf72 RNA foci as therapy for ALS and frontotemporal degeneration.
Lagier-Tourenne C, Baughn M, Rigo F, Sun S, Liu P, Li HR, Jiang J, Watt AT, Chun S, Katz M, Qiu J, Sun Y, Ling SC, Zhu Q, Polymenidou M, Drenner K, Artates JW, McAlonis-Downes M, Markmiller S, Hutt KR, Pizzo DP, Cady J, Harms MB, Baloh RH, Vandenberg SR, Yeo GW, Fu XD, Bennett CF, Cleveland DW, Ravits J
Proceedings of the National Academy of Sciences of the United States of America 2013 Nov 19;110(47):E4530-9
Proceedings of the National Academy of Sciences of the United States of America 2013 Nov 19;110(47):E4530-9
RNA binding mediates neurotoxicity in the transgenic Drosophila model of TDP-43 proteinopathy.
Ihara R, Matsukawa K, Nagata Y, Kunugi H, Tsuji S, Chihara T, Kuranaga E, Miura M, Wakabayashi T, Hashimoto T, Iwatsubo T
Human molecular genetics 2013 Nov 15;22(22):4474-84
Human molecular genetics 2013 Nov 15;22(22):4474-84
C9ORF72 expansion in a family with bipolar disorder.
Meisler MH, Grant AE, Jones JM, Lenk GM, He F, Todd PK, Kamali M, Albin RL, Lieberman AP, Langenecker SA, McInnis MG
Bipolar disorders 2013 May;15(3):326-32
Bipolar disorders 2013 May;15(3):326-32
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration.
Iguchi Y, Katsuno M, Niwa J, Takagi S, Ishigaki S, Ikenaka K, Kawai K, Watanabe H, Yamanaka K, Takahashi R, Misawa H, Sasaki S, Tanaka F, Sobue G
Brain : a journal of neurology 2013 May;136(Pt 5):1371-82
Brain : a journal of neurology 2013 May;136(Pt 5):1371-82
Extensive aggregation of α-synuclein and tau in juvenile-onset neuroaxonal dystrophy: an autopsied individual with a novel mutation in the PLA2G6 gene-splicing site.
Riku Y, Ikeuchi T, Yoshino H, Mimuro M, Mano K, Goto Y, Hattori N, Sobue G, Yoshida M
Acta neuropathologica communications 2013 May 9;1:12
Acta neuropathologica communications 2013 May 9;1:12
Expanded GGGGCC repeat RNA associated with amyotrophic lateral sclerosis and frontotemporal dementia causes neurodegeneration.
Xu Z, Poidevin M, Li X, Li Y, Shu L, Nelson DL, Li H, Hales CM, Gearing M, Wingo TS, Jin P
Proceedings of the National Academy of Sciences of the United States of America 2013 May 7;110(19):7778-83
Proceedings of the National Academy of Sciences of the United States of America 2013 May 7;110(19):7778-83
Aberrant assembly of RNA recognition motif 1 links to pathogenic conversion of TAR DNA-binding protein of 43 kDa (TDP-43).
Shodai A, Morimura T, Ido A, Uchida T, Ayaki T, Takahashi R, Kitazawa S, Suzuki S, Shirouzu M, Kigawa T, Muto Y, Yokoyama S, Takahashi R, Kitahara R, Ito H, Fujiwara N, Urushitani M
The Journal of biological chemistry 2013 May 24;288(21):14886-905
The Journal of biological chemistry 2013 May 24;288(21):14886-905
DNAJB6 myopathy in an Asian cohort and cytoplasmic/nuclear inclusions.
Sato T, Hayashi YK, Oya Y, Kondo T, Sugie K, Kaneda D, Houzen H, Yabe I, Sasaki H, Noguchi S, Nonaka I, Osawa M, Nishino I
Neuromuscular disorders : NMD 2013 Mar;23(3):269-76
Neuromuscular disorders : NMD 2013 Mar;23(3):269-76
Making the diagnosis of frontotemporal lobar degeneration.
Bigio EH
Archives of pathology & laboratory medicine 2013 Mar;137(3):314-25
Archives of pathology & laboratory medicine 2013 Mar;137(3):314-25
The truncated C-terminal RNA recognition motif of TDP-43 protein plays a key role in forming proteinaceous aggregates.
Wang YT, Kuo PH, Chiang CH, Liang JR, Chen YR, Wang S, Shen JC, Yuan HS
The Journal of biological chemistry 2013 Mar 29;288(13):9049-57
The Journal of biological chemistry 2013 Mar 29;288(13):9049-57
Mutations in prion-like domains in hnRNPA2B1 and hnRNPA1 cause multisystem proteinopathy and ALS.
Kim HJ, Kim NC, Wang YD, Scarborough EA, Moore J, Diaz Z, MacLea KS, Freibaum B, Li S, Molliex A, Kanagaraj AP, Carter R, Boylan KB, Wojtas AM, Rademakers R, Pinkus JL, Greenberg SA, Trojanowski JQ, Traynor BJ, Smith BN, Topp S, Gkazi AS, Miller J, Shaw CE, Kottlors M, Kirschner J, Pestronk A, Li YR, Ford AF, Gitler AD, Benatar M, King OD, Kimonis VE, Ross ED, Weihl CC, Shorter J, Taylor JP
Nature 2013 Mar 28;495(7442):467-73
Nature 2013 Mar 28;495(7442):467-73
Exome sequencing identifies titin mutations causing hereditary myopathy with early respiratory failure (HMERF) in families of diverse ethnic origins.
Toro C, Olivé M, Dalakas MC, Sivakumar K, Bilbao JM, Tyndel F, Vidal N, Farrero E, Sambuughin N, Goldfarb LG
BMC neurology 2013 Mar 20;13:29
BMC neurology 2013 Mar 20;13:29
Astrocyte pathology and the absence of non-cell autonomy in an induced pluripotent stem cell model of TDP-43 proteinopathy.
Serio A, Bilican B, Barmada SJ, Ando DM, Zhao C, Siller R, Burr K, Haghi G, Story D, Nishimura AL, Carrasco MA, Phatnani HP, Shum C, Wilmut I, Maniatis T, Shaw CE, Finkbeiner S, Chandran S
Proceedings of the National Academy of Sciences of the United States of America 2013 Mar 19;110(12):4697-702
Proceedings of the National Academy of Sciences of the United States of America 2013 Mar 19;110(12):4697-702
Luminescent conjugated oligothiophenes for sensitive fluorescent assignment of protein inclusion bodies.
Klingstedt T, Blechschmidt C, Nogalska A, Prokop S, Häggqvist B, Danielsson O, Engel WK, Askanas V, Heppner FL, Nilsson KP
Chembiochem : a European journal of chemical biology 2013 Mar 18;14(5):607-16
Chembiochem : a European journal of chemical biology 2013 Mar 18;14(5):607-16
The C9orf72 GGGGCC repeat is translated into aggregating dipeptide-repeat proteins in FTLD/ALS.
Mori K, Weng SM, Arzberger T, May S, Rentzsch K, Kremmer E, Schmid B, Kretzschmar HA, Cruts M, Van Broeckhoven C, Haass C, Edbauer D
Science (New York, N.Y.) 2013 Mar 15;339(6125):1335-8
Science (New York, N.Y.) 2013 Mar 15;339(6125):1335-8
Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration.
Onyike CU, Pletnikova O, Sloane KL, Sullivan C, Troncoso JC, Rabins PV
Dementia & neuropsychologia 2013 Mar 1;7(1):83-87
Dementia & neuropsychologia 2013 Mar 1;7(1):83-87
Progranulin mutations as risk factors for Alzheimer disease.
Perry DC, Lehmann M, Yokoyama JS, Karydas A, Lee JJ, Coppola G, Grinberg LT, Geschwind D, Seeley WW, Miller BL, Rosen H, Rabinovici G
JAMA neurology 2013 Jun;70(6):774-8
JAMA neurology 2013 Jun;70(6):774-8
α-Synuclein coaggregation in familial amyotrophic lateral sclerosis with SOD1 gene mutation.
Takei Y, Oguchi K, Koshihara H, Hineno A, Nakamura A, Ohara S
Human pathology 2013 Jun;44(6):1171-6
Human pathology 2013 Jun;44(6):1171-6
Optineurin is potentially associated with TDP-43 and involved in the pathogenesis of inclusion body myositis.
Yamashita S, Kimura E, Tawara N, Sakaguchi H, Nakama T, Maeda Y, Hirano T, Uchino M, Ando Y
Neuropathology and applied neurobiology 2013 Jun;39(4):406-16
Neuropathology and applied neurobiology 2013 Jun;39(4):406-16
Laminar distribution of the pathological changes in sporadic frontotemporal lobar degeneration with transactive response (TAR) DNA-binding protein of 43 kDa (TDP-43) proteinopathy: a quantitative study using polynomial curve fitting.
Armstrong RA, Hamilton RL, Mackenzie IR, Hedreen J, Cairns NJ
Neuropathology and applied neurobiology 2013 Jun;39(4):335-47
Neuropathology and applied neurobiology 2013 Jun;39(4):335-47
Ubiquilin-2 (UBQLN2) binds with high affinity to the C-terminal region of TDP-43 and modulates TDP-43 levels in H4 cells: characterization of inhibition by nucleic acids and 4-aminoquinolines.
Cassel JA, Reitz AB
Biochimica et biophysica acta 2013 Jun;1834(6):964-71
Biochimica et biophysica acta 2013 Jun;1834(6):964-71
Comment on "Drug screening for ALS using patient-specific induced pluripotent stem cells".
Bilican B, Serio A, Barmada SJ, Nishimura AL, Sullivan GJ, Carrasco M, Phatnani HP, Puddifoot CA, Story D, Fletcher J, Park IH, Friedman BA, Daley GQ, Wyllie DJ, Hardingham GE, Wilmut I, Finkbeiner S, Maniatis T, Shaw CE, Chandran S
Science translational medicine 2013 Jun 5;5(188):188le2
Science translational medicine 2013 Jun 5;5(188):188le2
Eukaryotic stress granules are cleared by autophagy and Cdc48/VCP function.
Buchan JR, Kolaitis RM, Taylor JP, Parker R
Cell 2013 Jun 20;153(7):1461-74
Cell 2013 Jun 20;153(7):1461-74
Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.
Hewamadduma CA, Grierson AJ, Ma TP, Pan L, Moens CB, Ingham PW, Ramesh T, Shaw PJ
Human molecular genetics 2013 Jun 15;22(12):2376-86
Human molecular genetics 2013 Jun 15;22(12):2376-86
Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo.
Vaccaro A, Patten SA, Aggad D, Julien C, Maios C, Kabashi E, Drapeau P, Parker JA
Neurobiology of disease 2013 Jul;55:64-75
Neurobiology of disease 2013 Jul;55:64-75
Selective forelimb impairment in rats expressing a pathological TDP-43 25 kDa C-terminal fragment to mimic amyotrophic lateral sclerosis.
Dayton RD, Gitcho MA, Orchard EA, Wilson JD, Wang DB, Cain CD, Johnson JA, Zhang YJ, Petrucelli L, Mathis JM, Klein RL
Molecular therapy : the journal of the American Society of Gene Therapy 2013 Jul;21(7):1324-34
Molecular therapy : the journal of the American Society of Gene Therapy 2013 Jul;21(7):1324-34
Robust cytoplasmic accumulation of phosphorylated TDP-43 in transgenic models of tauopathy.
Clippinger AK, D'Alton S, Lin WL, Gendron TF, Howard J, Borchelt DR, Cannon A, Carlomagno Y, Chakrabarty P, Cook C, Golde TE, Levites Y, Ranum L, Schultheis PJ, Xu G, Petrucelli L, Sahara N, Dickson DW, Giasson B, Lewis J
Acta neuropathologica 2013 Jul;126(1):39-50
Acta neuropathologica 2013 Jul;126(1):39-50
The long non-coding RNA nuclear-enriched abundant transcript 1_2 induces paraspeckle formation in the motor neuron during the early phase of amyotrophic lateral sclerosis.
Nishimoto Y, Nakagawa S, Hirose T, Okano HJ, Takao M, Shibata S, Suyama S, Kuwako K, Imai T, Murayama S, Suzuki N, Okano H
Molecular brain 2013 Jul 8;6:31
Molecular brain 2013 Jul 8;6:31
Neuron enriched nuclear proteome isolated from human brain.
Dammer EB, Duong DM, Diner I, Gearing M, Feng Y, Lah JJ, Levey AI, Seyfried NT
Journal of proteome research 2013 Jul 5;12(7):3193-206
Journal of proteome research 2013 Jul 5;12(7):3193-206
The pathogenesis of cingulate atrophy in behavioral variant frontotemporal dementia and Alzheimer's disease.
Tan RH, Pok K, Wong S, Brooks D, Halliday GM, Kril JJ
Acta neuropathologica communications 2013 Jul 5;1:30
Acta neuropathologica communications 2013 Jul 5;1:30
Functional genomic screen of human stem cell differentiation reveals pathways involved in neurodevelopment and neurodegeneration.
Zhang Y, Schulz VP, Reed BD, Wang Z, Pan X, Mariani J, Euskirchen G, Snyder MP, Vaccarino FM, Ivanova N, Weissman SM, Szekely AM
Proceedings of the National Academy of Sciences of the United States of America 2013 Jul 23;110(30):12361-6
Proceedings of the National Academy of Sciences of the United States of America 2013 Jul 23;110(30):12361-6
Prion-like properties of pathological TDP-43 aggregates from diseased brains.
Nonaka T, Masuda-Suzukake M, Arai T, Hasegawa Y, Akatsu H, Obi T, Yoshida M, Murayama S, Mann DM, Akiyama H, Hasegawa M
Cell reports 2013 Jul 11;4(1):124-34
Cell reports 2013 Jul 11;4(1):124-34
Classification of FTLD-TDP cases into pathological subtypes using antibodies against phosphorylated and non-phosphorylated TDP43.
Tan RH, Shepherd CE, Kril JJ, McCann H, McGeachie A, McGinley C, Affleck A, Halliday GM
Acta neuropathologica communications 2013 Jul 10;1(1):33
Acta neuropathologica communications 2013 Jul 10;1(1):33
Comparative utility of LC3, p62 and TDP-43 immunohistochemistry in differentiation of inclusion body myositis from polymyositis and related inflammatory myopathies.
Hiniker A, Daniels BH, Lee HS, Margeta M
Acta neuropathologica communications 2013 Jul 1;1:29
Acta neuropathologica communications 2013 Jul 1;1:29
Essential tremor followed by progressive supranuclear palsy: postmortem reports of 11 patients.
Louis ED, Babij R, Ma K, Cortés E, Vonsattel JP
Journal of neuropathology and experimental neurology 2013 Jan;72(1):8-17
Journal of neuropathology and experimental neurology 2013 Jan;72(1):8-17
Expression of myogenic regulatory factors and myo-endothelial remodeling in sporadic inclusion body myositis.
Wanschitz JV, Dubourg O, Lacene E, Fischer MB, Höftberger R, Budka H, Romero NB, Eymard B, Herson S, Butler-Browne GS, Voit T, Benveniste O
Neuromuscular disorders : NMD 2013 Jan;23(1):75-83
Neuromuscular disorders : NMD 2013 Jan;23(1):75-83
African ancestry protects against Alzheimer's disease-related neuropathology.
Schlesinger D, Grinberg LT, Alba JG, Naslavsky MS, Licinio L, Farfel JM, Suemoto CK, de Lucena Ferretti RE, Leite RE, de Andrade MP, dos Santos AC, Brentani H, Pasqualucci CA, Nitrini R, Jacob-Filho W, Zatz M, Brazilian Aging Brain Study Group
Molecular psychiatry 2013 Jan;18(1):79-85
Molecular psychiatry 2013 Jan;18(1):79-85
Patterns of striatal degeneration in frontotemporal dementia.
Halabi C, Halabi A, Dean DL, Wang PN, Boxer AL, Trojanowski JQ, Dearmond SJ, Miller BL, Kramer JH, Seeley WW
Alzheimer disease and associated disorders 2013 Jan-Mar;27(1):74-83
Alzheimer disease and associated disorders 2013 Jan-Mar;27(1):74-83
Widespread aggregation of mutant VAPB associated with ALS does not cause motor neuron degeneration or modulate mutant SOD1 aggregation and toxicity in mice.
Qiu L, Qiao T, Beers M, Tan W, Wang H, Yang B, Xu Z
Molecular neurodegeneration 2013 Jan 3;8:1
Molecular neurodegeneration 2013 Jan 3;8:1
Glucocorticoids exacerbate cognitive deficits in TDP-25 transgenic mice via a glutathione-mediated mechanism: implications for aging, stress and TDP-43 proteinopathies.
Caccamo A, Medina DX, Oddo S
The Journal of neuroscience : the official journal of the Society for Neuroscience 2013 Jan 16;33(3):906-13
The Journal of neuroscience : the official journal of the Society for Neuroscience 2013 Jan 16;33(3):906-13
New NBIA subtype: genetic, clinical, pathologic, and radiographic features of MPAN.
Hogarth P, Gregory A, Kruer MC, Sanford L, Wagoner W, Natowicz MR, Egel RT, Subramony SH, Goldman JG, Berry-Kravis E, Foulds NC, Hammans SR, Desguerre I, Rodriguez D, Wilson C, Diedrich A, Green S, Tran H, Reese L, Woltjer RL, Hayflick SJ
Neurology 2013 Jan 15;80(3):268-75
Neurology 2013 Jan 15;80(3):268-75
Involvement of endoplasmic reticulum stress in tauopathy.
Sakagami Y, Kudo T, Tanimukai H, Kanayama D, Omi T, Horiguchi K, Okochi M, Imaizumi K, Takeda M
Biochemical and biophysical research communications 2013 Jan 11;430(2):500-4
Biochemical and biophysical research communications 2013 Jan 11;430(2):500-4
Premature death of TDP-43 (A315T) transgenic mice due to gastrointestinal complications prior to development of full neurological symptoms of amyotrophic lateral sclerosis.
Esmaeili MA, Panahi M, Yadav S, Hennings L, Kiaei M
International journal of experimental pathology 2013 Feb;94(1):56-64
International journal of experimental pathology 2013 Feb;94(1):56-64
Spliceosome integrity is defective in the motor neuron diseases ALS and SMA.
Tsuiji H, Iguchi Y, Furuya A, Kataoka A, Hatsuta H, Atsuta N, Tanaka F, Hashizume Y, Akatsu H, Murayama S, Sobue G, Yamanaka K
EMBO molecular medicine 2013 Feb;5(2):221-34
EMBO molecular medicine 2013 Feb;5(2):221-34
Nuclear carrier and RNA-binding proteins in frontotemporal lobar degeneration associated with fused in sarcoma (FUS) pathological changes.
Davidson YS, Robinson AC, Hu Q, Mishra M, Baborie A, Jaros E, Perry RH, Cairns NJ, Richardson A, Gerhard A, Neary D, Snowden JS, Bigio EH, Mann DM
Neuropathology and applied neurobiology 2013 Feb;39(2):157-65
Neuropathology and applied neurobiology 2013 Feb;39(2):157-65
Regionally different immunoreactivity for Smurf2 and pSmad2/3 in TDP-43-positive inclusions of amyotrophic lateral sclerosis.
Nakamura M, Kaneko S, Wate R, Asayama S, Nakamura Y, Fujita K, Ito H, Kusaka H
Neuropathology and applied neurobiology 2013 Feb;39(2):144-56
Neuropathology and applied neurobiology 2013 Feb;39(2):144-56
Pantothenate kinase-associated neurodegeneration is not a synucleinopathy.
Li A, Paudel R, Johnson R, Courtney R, Lees AJ, Holton JL, Hardy J, Revesz T, Houlden H
Neuropathology and applied neurobiology 2013 Feb;39(2):121-31
Neuropathology and applied neurobiology 2013 Feb;39(2):121-31
A fatal neuromuscular disease in an adult patient after poliomyelitis in early childhood: consideration of the pathology of post-polio syndrome.
Kosaka T, Kuroha Y, Tada M, Hasegawa A, Tani T, Matsubara N, Koike R, Toyoshima Y, Takahashi H
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Feb;33(1):93-101
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Feb;33(1):93-101
Atypical FTLD-FUS associated with ALS-TDP: a case report.
Kobayashi Z, Arai T, Yokota O, Tsuchiya K, Hosokawa M, Oshima K, Niizato K, Akiyama H, Mizusawa H
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Feb;33(1):83-6
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Feb;33(1):83-6
Degeneration of serotonergic neurons in amyotrophic lateral sclerosis: a link to spasticity.
Dentel C, Palamiuc L, Henriques A, Lannes B, Spreux-Varoquaux O, Gutknecht L, René F, Echaniz-Laguna A, Gonzalez de Aguilar JL, Lesch KP, Meininger V, Loeffler JP, Dupuis L
Brain : a journal of neurology 2013 Feb;136(Pt 2):483-93
Brain : a journal of neurology 2013 Feb;136(Pt 2):483-93
Oligodendrocyte dysfunction in the pathogenesis of amyotrophic lateral sclerosis.
Philips T, Bento-Abreu A, Nonneman A, Haeck W, Staats K, Geelen V, Hersmus N, Küsters B, Van Den Bosch L, Van Damme P, Richardson WD, Robberecht W
Brain : a journal of neurology 2013 Feb;136(Pt 2):471-82
Brain : a journal of neurology 2013 Feb;136(Pt 2):471-82
Corticospinal tract degeneration associated with TDP-43 type C pathology and semantic dementia.
Josephs KA, Whitwell JL, Murray ME, Parisi JE, Graff-Radford NR, Knopman DS, Boeve BF, Senjem ML, Rademakers R, Jack CR Jr, Petersen RC, Dickson DW
Brain : a journal of neurology 2013 Feb;136(Pt 2):455-70
Brain : a journal of neurology 2013 Feb;136(Pt 2):455-70
Parkin ubiquitinates Tar-DNA binding protein-43 (TDP-43) and promotes its cytosolic accumulation via interaction with histone deacetylase 6 (HDAC6).
Hebron ML, Lonskaya I, Sharpe K, Weerasinghe PP, Algarzae NK, Shekoyan AR, Moussa CE
The Journal of biological chemistry 2013 Feb 8;288(6):4103-15
The Journal of biological chemistry 2013 Feb 8;288(6):4103-15
Identification of in vivo, conserved, TAF15 RNA binding sites reveals the impact of TAF15 on the neuronal transcriptome.
Ibrahim F, Maragkakis M, Alexiou P, Maronski MA, Dichter MA, Mourelatos Z
Cell reports 2013 Feb 21;3(2):301-8
Cell reports 2013 Feb 21;3(2):301-8
Reduction of polyglutamine toxicity by TDP-43, FUS and progranulin in Huntington's disease models.
Tauffenberger A, Chitramuthu BP, Bateman A, Bennett HP, Parker JA
Human molecular genetics 2013 Feb 15;22(4):782-94
Human molecular genetics 2013 Feb 15;22(4):782-94
The frontotemporal lobar degeneration risk factor, TMEM106B, regulates lysosomal morphology and function.
Brady OA, Zheng Y, Murphy K, Huang M, Hu F
Human molecular genetics 2013 Feb 15;22(4):685-95
Human molecular genetics 2013 Feb 15;22(4):685-95
A unique mouse model for investigating the properties of amyotrophic lateral sclerosis-associated protein TDP-43, by in utero electroporation.
Akamatsu M, Takuma H, Yamashita T, Okada T, Keino-Masu K, Ishii K, Kwak S, Masu M, Tamaoka A
Neuroscience research 2013 Dec;77(4):234-41
Neuroscience research 2013 Dec;77(4):234-41
A clinicopathological and genetic study of sporadic diffuse leukoencephalopathy with spheroids: a report of two cases.
Kimura T, Ishizawa K, Mitsufuji T, Abe T, Nakazato Y, Yoshida K, Sasaki A, Araki N
Neuropathology and applied neurobiology 2013 Dec;39(7):837-43
Neuropathology and applied neurobiology 2013 Dec;39(7):837-43
Accumulation of phosphorylated TDP-43 in the CNS of a patient with Cockayne syndrome.
Sakurai A, Makioka K, Fukuda T, Takatama M, Okamoto K
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):673-7
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):673-7
Valosin-containing protein immunoreactivity in tauopathies, synucleinopathies, polyglutamine diseases and intranuclear inclusion body disease.
Mori F, Tanji K, Toyoshima Y, Sasaki H, Yoshida M, Kakita A, Takahashi H, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):637-44
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):637-44
Increased number of astrocytes and macrophages/microglial cells in the corpus callosum in amyotrophic lateral sclerosis.
Sugiyama M, Takao M, Hatsuta H, Funabe S, Ito S, Obi T, Tanaka F, Kuroiwa Y, Murayama S
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):591-9
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Dec;33(6):591-9
Altered astrocytic expression of TDP-43 does not influence motor neuron survival.
Haidet-Phillips AM, Gross SK, Williams T, Tuteja A, Sherman A, Ko M, Jeong YH, Wong PC, Maragakis NJ
Experimental neurology 2013 Dec;250:250-9
Experimental neurology 2013 Dec;250:250-9
Neurocognitive speed associates with frontotemporal lobar degeneration TDP-43 subtypes.
Dean PM, Smith GE, Parisi JE, Dickson DW, Petersen RC, Josephs KA
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2013 Dec;20(12):1737-41
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2013 Dec;20(12):1737-41
Cell number changes in Alzheimer's disease relate to dementia, not to plaques and tangles.
Andrade-Moraes CH, Oliveira-Pinto AV, Castro-Fonseca E, da Silva CG, Guimarães DM, Szczupak D, Parente-Bruno DR, Carvalho LR, Polichiso L, Gomes BV, Oliveira LM, Rodriguez RD, Leite RE, Ferretti-Rebustini RE, Jacob-Filho W, Pasqualucci CA, Grinberg LT, Lent R
Brain : a journal of neurology 2013 Dec;136(Pt 12):3738-52
Brain : a journal of neurology 2013 Dec;136(Pt 12):3738-52
Dipeptide repeat protein pathology in C9ORF72 mutation cases: clinico-pathological correlations.
Mackenzie IR, Arzberger T, Kremmer E, Troost D, Lorenzl S, Mori K, Weng SM, Haass C, Kretzschmar HA, Edbauer D, Neumann M
Acta neuropathologica 2013 Dec;126(6):859-79
Acta neuropathologica 2013 Dec;126(6):859-79
Pathological features of FTLD-FUS in a Japanese population: analyses of nine cases.
Kobayashi Z, Kawakami I, Arai T, Yokota O, Tsuchiya K, Kondo H, Shimomura Y, Haga C, Aoki N, Hasegawa M, Hosokawa M, Oshima K, Niizato K, Ishizu H, Terada S, Onaya M, Ikeda M, Oyanagi K, Nakano I, Murayama S, Akiyama H, Mizusawa H
Journal of the neurological sciences 2013 Dec 15;335(1-2):89-95
Journal of the neurological sciences 2013 Dec 15;335(1-2):89-95
Hexanucleotide repeats in ALS/FTD form length-dependent RNA foci, sequester RNA binding proteins, and are neurotoxic.
Lee YB, Chen HJ, Peres JN, Gomez-Deza J, Attig J, Stalekar M, Troakes C, Nishimura AL, Scotter EL, Vance C, Adachi Y, Sardone V, Miller JW, Smith BN, Gallo JM, Ule J, Hirth F, Rogelj B, Houart C, Shaw CE
Cell reports 2013 Dec 12;5(5):1178-86
Cell reports 2013 Dec 12;5(5):1178-86
Genetic CJD with a novel E200G mutation in the prion protein gene and comparison with E200K mutation cases.
Kim MO, Cali I, Oehler A, Fong JC, Wong K, See T, Katz JS, Gambetti P, Bettcher BM, Dearmond SJ, Geschwind MD
Acta neuropathologica communications 2013 Dec 12;1:80
Acta neuropathologica communications 2013 Dec 12;1:80
Overexpression of ALS-associated p.M337V human TDP-43 in mice worsens disease features compared to wild-type human TDP-43 mice.
Janssens J, Wils H, Kleinberger G, Joris G, Cuijt I, Ceuterick-de Groote C, Van Broeckhoven C, Kumar-Singh S
Molecular neurobiology 2013 Aug;48(1):22-35
Molecular neurobiology 2013 Aug;48(1):22-35
Lack of unique neuropathology in amyotrophic lateral sclerosis associated with p.K54E angiogenin (ANG) mutation.
Kirby J, Highley JR, Cox L, Goodall EF, Hewitt C, Hartley JA, Hollinger HC, Fox M, Ince PG, McDermott CJ, Shaw PJ
Neuropathology and applied neurobiology 2013 Aug;39(5):562-71
Neuropathology and applied neurobiology 2013 Aug;39(5):562-71
Surgical pathology of epilepsy-associated non-neoplastic cerebral lesions: a brief introduction with special reference to hippocampal sclerosis and focal cortical dysplasia.
Miyata H, Hori T, Vinters HV
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Aug;33(4):442-58
Neuropathology : official journal of the Japanese Society of Neuropathology 2013 Aug;33(4):442-58
Reducing TDP-43 aggregation does not prevent its cytotoxicity.
Liu R, Yang G, Nonaka T, Arai T, Jia W, Cynader MS
Acta neuropathologica communications 2013 Aug 9;1:49
Acta neuropathologica communications 2013 Aug 9;1:49
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
Zhang YJ, Caulfield T, Xu YF, Gendron TF, Hubbard J, Stetler C, Sasaguri H, Whitelaw EC, Cai S, Lee WC, Petrucelli L
Human molecular genetics 2013 Aug 1;22(15):3112-22
Human molecular genetics 2013 Aug 1;22(15):3112-22
Japanese amyotrophic lateral sclerosis patients with GGGGCC hexanucleotide repeat expansion in C9ORF72.
Konno T, Shiga A, Tsujino A, Sugai A, Kato T, Kanai K, Yokoseki A, Eguchi H, Kuwabara S, Nishizawa M, Takahashi H, Onodera O
Journal of neurology, neurosurgery, and psychiatry 2013 Apr;84(4):398-401
Journal of neurology, neurosurgery, and psychiatry 2013 Apr;84(4):398-401
G51D α-synuclein mutation causes a novel parkinsonian-pyramidal syndrome.
Lesage S, Anheim M, Letournel F, Bousset L, Honoré A, Rozas N, Pieri L, Madiona K, Dürr A, Melki R, Verny C, Brice A, French Parkinson's Disease Genetics Study Group
Annals of neurology 2013 Apr;73(4):459-71
Annals of neurology 2013 Apr;73(4):459-71
Welander distal myopathy caused by an ancient founder mutation in TIA1 associated with perturbed splicing.
Klar J, Sobol M, Melberg A, Mäbert K, Ameur A, Johansson AC, Feuk L, Entesarian M, Orlén H, Casar-Borota O, Dahl N
Human mutation 2013 Apr;34(4):572-7
Human mutation 2013 Apr;34(4):572-7
Co-occurrence of TDP-43 mislocalization with reduced activity of an RNA editing enzyme, ADAR2, in aged mouse motor neurons.
Hideyama T, Teramoto S, Hachiga K, Yamashita T, Kwak S
PloS one 2012;7(8):e43469
PloS one 2012;7(8):e43469
Alteration of POLDIP3 splicing associated with loss of function of TDP-43 in tissues affected with ALS.
Shiga A, Ishihara T, Miyashita A, Kuwabara M, Kato T, Watanabe N, Yamahira A, Kondo C, Yokoseki A, Takahashi M, Kuwano R, Kakita A, Nishizawa M, Takahashi H, Onodera O
PloS one 2012;7(8):e43120
PloS one 2012;7(8):e43120
Inhibition of TDP-43 accumulation by bis(thiosemicarbazonato)-copper complexes.
Parker SJ, Meyerowitz J, James JL, Liddell JR, Nonaka T, Hasegawa M, Kanninen KM, Lim S, Paterson BM, Donnelly PS, Crouch PJ, White AR
PloS one 2012;7(8):e42277
PloS one 2012;7(8):e42277
Methylene blue protects against TDP-43 and FUS neuronal toxicity in C. elegans and D. rerio.
Vaccaro A, Patten SA, Ciura S, Maios C, Therrien M, Drapeau P, Kabashi E, Parker JA
PloS one 2012;7(7):e42117
PloS one 2012;7(7):e42117
Coaggregation of RNA-binding proteins in a model of TDP-43 proteinopathy with selective RGG motif methylation and a role for RRM1 ubiquitination.
Dammer EB, Fallini C, Gozal YM, Duong DM, Rossoll W, Xu P, Lah JJ, Levey AI, Peng J, Bassell GJ, Seyfried NT
PloS one 2012;7(6):e38658
PloS one 2012;7(6):e38658
TDP-43 identified from a genome wide RNAi screen for SOD1 regulators.
Somalinga BR, Day CE, Wei S, Roth MG, Thomas PJ
PloS one 2012;7(4):e35818
PloS one 2012;7(4):e35818
Iron accumulation in deep cortical layers accounts for MRI signal abnormalities in ALS: correlating 7 tesla MRI and pathology.
Kwan JY, Jeong SY, Van Gelderen P, Deng HX, Quezado MM, Danielian LE, Butman JA, Chen L, Bayat E, Russell J, Siddique T, Duyn JH, Rouault TA, Floeter MK
PloS one 2012;7(4):e35241
PloS one 2012;7(4):e35241
Aberrant localization of FUS and TDP43 is associated with misfolding of SOD1 in amyotrophic lateral sclerosis.
Pokrishevsky E, Grad LI, Yousefi M, Wang J, Mackenzie IR, Cashman NR
PloS one 2012;7(4):e35050
PloS one 2012;7(4):e35050
Mutant TDP-43 and FUS cause age-dependent paralysis and neurodegeneration in C. elegans.
Vaccaro A, Tauffenberger A, Aggad D, Rouleau G, Drapeau P, Parker JA
PloS one 2012;7(2):e31321
PloS one 2012;7(2):e31321
Conserved acidic amino acid residues in a second RNA recognition motif regulate assembly and function of TDP-43.
Shodai A, Ido A, Fujiwara N, Ayaki T, Morimura T, Oono M, Uchida T, Takahashi R, Ito H, Urushitani M
PloS one 2012;7(12):e52776
PloS one 2012;7(12):e52776
Clinical, neuropathological, and genetic characteristics of the novel IVS9+1delG GRN mutation in a patient with frontotemporal dementia.
Taipa R, Tuna A, Damásio J, Pinto PS, Cavaco S, Pereira S, Milterberger-Miltenyi G, Galimberti D, Melo-Pires M
Journal of Alzheimer's disease : JAD 2012;30(1):83-90
Journal of Alzheimer's disease : JAD 2012;30(1):83-90
Characterization of staufen1 ribonucleoproteins by mass spectrometry and biochemical analyses reveal the presence of diverse host proteins associated with human immunodeficiency virus type 1.
Milev MP, Ravichandran M, Khan MF, Schriemer DC, Mouland AJ
Frontiers in microbiology 2012;3:367
Frontiers in microbiology 2012;3:367
Lack of miRNA Misregulation at Early Pathological Stages in Drosophila Neurodegenerative Disease Models.
Reinhardt A, Feuillette S, Cassar M, Callens C, Thomassin H, Birman S, Lecourtois M, Antoniewski C, Tricoire H
Frontiers in genetics 2012;3:226
Frontiers in genetics 2012;3:226
A role for calpain-dependent cleavage of TDP-43 in amyotrophic lateral sclerosis pathology.
Yamashita T, Hideyama T, Hachiga K, Teramoto S, Takano J, Iwata N, Saido TC, Kwak S
Nature communications 2012;3:1307
Nature communications 2012;3:1307
Structural and molecular insights into the mechanism of action of human angiogenin-ALS variants in neurons.
Thiyagarajan N, Ferguson R, Subramanian V, Acharya KR
Nature communications 2012;3:1121
Nature communications 2012;3:1121
Widespread binding of FUS along nascent RNA regulates alternative splicing in the brain.
Rogelj B, Easton LE, Bogu GK, Stanton LW, Rot G, Curk T, Zupan B, Sugimoto Y, Modic M, Haberman N, Tollervey J, Fujii R, Takumi T, Shaw CE, Ule J
Scientific reports 2012;2:603
Scientific reports 2012;2:603
FUS/TLS-immunoreactive neuronal and glial cell inclusions increase with disease duration in familial amyotrophic lateral sclerosis with an R521C FUS/TLS mutation.
Suzuki N, Kato S, Kato M, Warita H, Mizuno H, Kato M, Shimakura N, Akiyama H, Kobayashi Z, Konno H, Aoki M
Journal of neuropathology and experimental neurology 2012 Sep;71(9):779-88
Journal of neuropathology and experimental neurology 2012 Sep;71(9):779-88
A novel TARDBP insertion/deletion mutation in the flail arm variant of amyotrophic lateral sclerosis.
Solski JA, Yang S, Nicholson GA, Luquin N, Williams KL, Fernando R, Pamphlett R, Blair IP
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Sep;13(5):465-70
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Sep;13(5):465-70
TDP-43 plasma levels are higher in amyotrophic lateral sclerosis.
Verstraete E, Kuiperij HB, van Blitterswijk MM, Veldink JH, Schelhaas HJ, van den Berg LH, Verbeek MM
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Sep;13(5):446-51
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Sep;13(5):446-51
UBQLN2/ubiquilin 2 mutation and pathology in familial amyotrophic lateral sclerosis.
Williams KL, Warraich ST, Yang S, Solski JA, Fernando R, Rouleau GA, Nicholson GA, Blair IP
Neurobiology of aging 2012 Oct;33(10):2527.e3-10
Neurobiology of aging 2012 Oct;33(10):2527.e3-10
Benign hereditary chorea 2: pathological findings in an autopsy case.
Yoshida Y, Nunomura J, Shimohata T, Nanjo H, Miyata H
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Oct;32(5):557-65
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Oct;32(5):557-65
An MND/ALS phenotype associated with C9orf72 repeat expansion: abundant p62-positive, TDP-43-negative inclusions in cerebral cortex, hippocampus and cerebellum but without associated cognitive decline.
Troakes C, Maekawa S, Wijesekera L, Rogelj B, Siklós L, Bell C, Smith B, Newhouse S, Vance C, Johnson L, Hortobágyi T, Shatunov A, Al-Chalabi A, Leigh N, Shaw CE, King A, Al-Sarraj S
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Oct;32(5):505-14
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Oct;32(5):505-14
In vivo and post-mortem memory circuit integrity in frontotemporal dementia and Alzheimer's disease.
Hornberger M, Wong S, Tan R, Irish M, Piguet O, Kril J, Hodges JR, Halliday G
Brain : a journal of neurology 2012 Oct;135(Pt 10):3015-25
Brain : a journal of neurology 2012 Oct;135(Pt 10):3015-25
Glucose delays age-dependent proteotoxicity.
Tauffenberger A, Vaccaro A, Aulas A, Vande Velde C, Parker JA
Aging cell 2012 Oct;11(5):856-66
Aging cell 2012 Oct;11(5):856-66
Endosomal sorting related protein CHMP2B is localized in Lewy bodies and glial cytoplasmic inclusions in α-synucleinopathy.
Tanikawa S, Mori F, Tanji K, Kakita A, Takahashi H, Wakabayashi K
Neuroscience letters 2012 Oct 3;527(1):16-21
Neuroscience letters 2012 Oct 3;527(1):16-21
FUS-SMN protein interactions link the motor neuron diseases ALS and SMA.
Yamazaki T, Chen S, Yu Y, Yan B, Haertlein TC, Carrasco MA, Tapia JC, Zhai B, Das R, Lalancette-Hebert M, Sharma A, Chandran S, Sullivan G, Nishimura AL, Shaw CE, Gygi SP, Shneider NA, Maniatis T, Reed R
Cell reports 2012 Oct 25;2(4):799-806
Cell reports 2012 Oct 25;2(4):799-806
Induced pluripotent stem cell models of progranulin-deficient frontotemporal dementia uncover specific reversible neuronal defects.
Almeida S, Zhang Z, Coppola G, Mao W, Futai K, Karydas A, Geschwind MD, Tartaglia MC, Gao F, Gianni D, Sena-Esteves M, Geschwind DH, Miller BL, Farese RV Jr, Gao FB
Cell reports 2012 Oct 25;2(4):789-98
Cell reports 2012 Oct 25;2(4):789-98
Endogenous TDP-43, but not FUS, contributes to stress granule assembly via G3BP.
Aulas A, Stabile S, Vande Velde C
Molecular neurodegeneration 2012 Oct 24;7:54
Molecular neurodegeneration 2012 Oct 24;7:54
Expression of Fused in sarcoma mutations in mice recapitulates the neuropathology of FUS proteinopathies and provides insight into disease pathogenesis.
Verbeeck C, Deng Q, Dejesus-Hernandez M, Taylor G, Ceballos-Diaz C, Kocerha J, Golde T, Das P, Rademakers R, Dickson DW, Kukar T
Molecular neurodegeneration 2012 Oct 10;7:53
Molecular neurodegeneration 2012 Oct 10;7:53
Convergent transcription induces transcriptional gene silencing in fission yeast and mammalian cells.
Gullerova M, Proudfoot NJ
Nature structural & molecular biology 2012 Nov;19(11):1193-201
Nature structural & molecular biology 2012 Nov;19(11):1193-201
Divergent roles of ALS-linked proteins FUS/TLS and TDP-43 intersect in processing long pre-mRNAs.
Lagier-Tourenne C, Polymenidou M, Hutt KR, Vu AQ, Baughn M, Huelga SC, Clutario KM, Ling SC, Liang TY, Mazur C, Wancewicz E, Kim AS, Watt A, Freier S, Hicks GG, Donohue JP, Shiue L, Bennett CF, Ravits J, Cleveland DW, Yeo GW
Nature neuroscience 2012 Nov;15(11):1488-97
Nature neuroscience 2012 Nov;15(11):1488-97
Molecular analysis and biochemical classification of TDP-43 proteinopathy.
Tsuji H, Arai T, Kametani F, Nonaka T, Yamashita M, Suzukake M, Hosokawa M, Yoshida M, Hatsuta H, Takao M, Saito Y, Murayama S, Akiyama H, Hasegawa M, Mann DM, Tamaoka A
Brain : a journal of neurology 2012 Nov;135(Pt 11):3380-91
Brain : a journal of neurology 2012 Nov;135(Pt 11):3380-91
Coexistence of Huntington's disease and amyotrophic lateral sclerosis: a clinicopathologic study.
Tada M, Coon EA, Osmand AP, Kirby PA, Martin W, Wieler M, Shiga A, Shirasaki H, Tada M, Makifuchi T, Yamada M, Kakita A, Nishizawa M, Takahashi H, Paulson HL
Acta neuropathologica 2012 Nov;124(5):749-60
Acta neuropathologica 2012 Nov;124(5):749-60
The RNA-binding motif 45 (RBM45) protein accumulates in inclusion bodies in amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP) patients.
Collins M, Riascos D, Kovalik T, An J, Krupa K, Krupa K, Hood BL, Conrads TP, Renton AE, Traynor BJ, Bowser R
Acta neuropathologica 2012 Nov;124(5):717-32
Acta neuropathologica 2012 Nov;124(5):717-32
Long non-coding RNA gadd7 interacts with TDP-43 and regulates Cdk6 mRNA decay.
Liu X, Li D, Zhang W, Guo M, Zhan Q
The EMBO journal 2012 Nov 28;31(23):4415-27
The EMBO journal 2012 Nov 28;31(23):4415-27
An early onset progressive motor neuron disorder in Scyl1-deficient mice is associated with mislocalization of TDP-43.
Pelletier S, Gingras S, Howell S, Vogel P, Ihle JN
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Nov 21;32(47):16560-73
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Nov 21;32(47):16560-73
Minocycline-associated rimmed vacuolar myopathy in a patient with rheumatoid arthritis.
Bokuda K, Sugaya K, Tamura S, Miyamoto K, Matsubara S, Komori T
BMC neurology 2012 Nov 21;12:140
BMC neurology 2012 Nov 21;12:140
High-content RNAi screening identifies the Type 1 inositol triphosphate receptor as a modifier of TDP-43 localization and neurotoxicity.
Kim SH, Zhan L, Hanson KA, Tibbetts RS
Human molecular genetics 2012 Nov 15;21(22):4845-56
Human molecular genetics 2012 Nov 15;21(22):4845-56
Entorhinal cortical neurons are the primary targets of FUS mislocalization and ubiquitin aggregation in FUS transgenic rats.
Huang C, Tong J, Bi F, Wu Q, Huang B, Zhou H, Xia XG
Human molecular genetics 2012 Nov 1;21(21):4602-14
Human molecular genetics 2012 Nov 1;21(21):4602-14
Wild type TDP-43 induces neuro-inflammation and alters APP metabolism in lentiviral gene transfer models.
Herman AM, Khandelwal PJ, Rebeck GW, Moussa CE
Experimental neurology 2012 May;235(1):297-305
Experimental neurology 2012 May;235(1):297-305
First neuropathological description of a patient with Parkinson's disease and LRRK2 p.N1437H mutation.
Puschmann A, Englund E, Ross OA, Vilariño-Güell C, Lincoln SJ, Kachergus JM, Cobb SA, Törnqvist AL, Rehncrona S, Widner H, Wszolek ZK, Farrer MJ, Nilsson C
Parkinsonism & related disorders 2012 May;18(4):332-8
Parkinsonism & related disorders 2012 May;18(4):332-8
Essential tremor with ubiquitinated intranuclear inclusions and cerebellar degeneration.
Louis ED, Mazzoni P, Ma KJ, Moskowitz CB, Lawton A, Garber A, Vonsattel JP
Clinical neuropathology 2012 May-Jun;31(3):119-26
Clinical neuropathology 2012 May-Jun;31(3):119-26
TDP-43 and FUS RNA-binding proteins bind distinct sets of cytoplasmic messenger RNAs and differently regulate their post-transcriptional fate in motoneuron-like cells.
Colombrita C, Onesto E, Megiorni F, Pizzuti A, Baralle FE, Buratti E, Silani V, Ratti A
The Journal of biological chemistry 2012 May 4;287(19):15635-47
The Journal of biological chemistry 2012 May 4;287(19):15635-47
Quantitative analysis of the detergent-insoluble brain proteome in frontotemporal lobar degeneration using SILAC internal standards.
Seyfried NT, Gozal YM, Donovan LE, Herskowitz JH, Dammer EB, Xia Q, Ku L, Chang J, Duong DM, Rees HD, Cooper DS, Glass JD, Gearing M, Tansey MG, Lah JJ, Feng Y, Levey AI, Peng J
Journal of proteome research 2012 May 4;11(5):2721-38
Journal of proteome research 2012 May 4;11(5):2721-38
Exome sequencing reveals DNAJB6 mutations in dominantly-inherited myopathy.
Harms MB, Sommerville RB, Allred P, Bell S, Ma D, Cooper P, Lopate G, Pestronk A, Weihl CC, Baloh RH
Annals of neurology 2012 Mar;71(3):407-16
Annals of neurology 2012 Mar;71(3):407-16
Endogenous TDP-43 localized to stress granules can subsequently form protein aggregates.
Parker SJ, Meyerowitz J, James JL, Liddell JR, Crouch PJ, Kanninen KM, White AR
Neurochemistry international 2012 Mar;60(4):415-24
Neurochemistry international 2012 Mar;60(4):415-24
Oxidative stress induced by glutathione depletion reproduces pathological modifications of TDP-43 linked to TDP-43 proteinopathies.
Iguchi Y, Katsuno M, Takagi S, Ishigaki S, Niwa J, Hasegawa M, Tanaka F, Sobue G
Neurobiology of disease 2012 Mar;45(3):862-70
Neurobiology of disease 2012 Mar;45(3):862-70
TDP-43 regulates global translational yield by splicing of exon junction complex component SKAR.
Fiesel FC, Weber SS, Supper J, Zell A, Kahle PJ
Nucleic acids research 2012 Mar;40(6):2668-82
Nucleic acids research 2012 Mar;40(6):2668-82
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.
Uchida A, Sasaguri H, Kimura N, Tajiri M, Ohkubo T, Ono F, Sakaue F, Kanai K, Hirai T, Sano T, Shibuya K, Kobayashi M, Yamamoto M, Yokota S, Kubodera T, Tomori M, Sakaki K, Enomoto M, Hirai Y, Kumagai J, Yasutomi Y, Mochizuki H, Kuwabara S, Uchihara T, Mizusawa H, Yokota T
Brain : a journal of neurology 2012 Mar;135(Pt 3):833-46
Brain : a journal of neurology 2012 Mar;135(Pt 3):833-46
Progressive neuronal inclusion formation and axonal degeneration in CHMP2B mutant transgenic mice.
Ghazi-Noori S, Froud KE, Mizielinska S, Powell C, Smidak M, Fernandez de Marco M, O'Malley C, Farmer M, Parkinson N, Fisher EM, Asante EA, Brandner S, Collinge J, Isaacs AM
Brain : a journal of neurology 2012 Mar;135(Pt 3):819-32
Brain : a journal of neurology 2012 Mar;135(Pt 3):819-32
Clinical and pathological features of familial frontotemporal dementia caused by C9ORF72 mutation on chromosome 9p.
Hsiung GY, DeJesus-Hernandez M, Feldman HH, Sengdy P, Bouchard-Kerr P, Dwosh E, Butler R, Leung B, Fok A, Rutherford NJ, Baker M, Rademakers R, Mackenzie IR
Brain : a journal of neurology 2012 Mar;135(Pt 3):709-22
Brain : a journal of neurology 2012 Mar;135(Pt 3):709-22
Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations.
Snowden JS, Rollinson S, Thompson JC, Harris JM, Stopford CL, Richardson AM, Jones M, Gerhard A, Davidson YS, Robinson A, Gibbons L, Hu Q, DuPlessis D, Neary D, Mann DM, Pickering-Brown SM
Brain : a journal of neurology 2012 Mar;135(Pt 3):693-708
Brain : a journal of neurology 2012 Mar;135(Pt 3):693-708
Clinical and pathological features of amyotrophic lateral sclerosis caused by mutation in the C9ORF72 gene on chromosome 9p.
Stewart H, Rutherford NJ, Briemberg H, Krieger C, Cashman N, Fabros M, Baker M, Fok A, DeJesus-Hernandez M, Eisen A, Rademakers R, Mackenzie IR
Acta neuropathologica 2012 Mar;123(3):409-17
Acta neuropathologica 2012 Mar;123(3):409-17
Caenorhabditis elegans RNA-processing protein TDP-1 regulates protein homeostasis and life span.
Zhang T, Hwang HY, Hao H, Talbot C Jr, Wang J
The Journal of biological chemistry 2012 Mar 9;287(11):8371-82
The Journal of biological chemistry 2012 Mar 9;287(11):8371-82
Redox signalling directly regulates TDP-43 via cysteine oxidation and disulphide cross-linking.
Cohen TJ, Hwang AW, Unger T, Trojanowski JQ, Lee VM
The EMBO journal 2012 Mar 7;31(5):1241-52
The EMBO journal 2012 Mar 7;31(5):1241-52
Phosphorylated tau/amyloid beta 1-42 ratio in ventricular cerebrospinal fluid reflects outcome in idiopathic normal pressure hydrocephalus.
Patel S, Lee EB, Xie SX, Law A, Jackson EM, Arnold SE, Clark CM, Shaw LM, Grady MS, Trojanowski JQ, Hamilton RH
Fluids and barriers of the CNS 2012 Mar 23;9(1):7
Fluids and barriers of the CNS 2012 Mar 23;9(1):7
Cellular model of TAR DNA-binding protein 43 (TDP-43) aggregation based on its C-terminal Gln/Asn-rich region.
Budini M, Buratti E, Stuani C, Guarnaccia C, Romano V, De Conti L, Baralle FE
The Journal of biological chemistry 2012 Mar 2;287(10):7512-25
The Journal of biological chemistry 2012 Mar 2;287(10):7512-25
The abnormal processing of TDP-43 is not an upstream event of reduced ADAR2 activity in ALS motor neurons.
Yamashita T, Hideyama T, Teramoto S, Kwak S
Neuroscience research 2012 Jun;73(2):153-60
Neuroscience research 2012 Jun;73(2):153-60
Progressive nonfluent aphasia: a rare clinical subtype of FTLD-TDP in Japan.
Aoki N, Tsuchiya K, Kobayashi Z, Arai T, Togo T, Miyazaki H, Kondo H, Ishizu H, Uchikado H, Katsuse O, Hirayasu Y, Akiyama H
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):272-9
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):272-9
Abnormal tau deposition in neurons, but not in glial cells in the cerebral tissue surrounding arteriovenous malformation.
Kon T, Mori F, Tanji K, Miki Y, Tomiyama M, Baba M, Umehara Y, Kurotaki H, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):267-71
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):267-71
Neuropathology in classical and variant ataxia-telangiectasia.
Verhagen MM, Martin JJ, van Deuren M, Ceuterick-de Groote C, Weemaes CM, Kremer BH, Taylor MA, Willemsen MA, Lammens M
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):234-44
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Jun;32(3):234-44
Requirements for stress granule recruitment of fused in sarcoma (FUS) and TAR DNA-binding protein of 43 kDa (TDP-43).
Bentmann E, Neumann M, Tahirovic S, Rodde R, Dormann D, Haass C
The Journal of biological chemistry 2012 Jun 29;287(27):23079-94
The Journal of biological chemistry 2012 Jun 29;287(27):23079-94
Rodent models of TDP-43: recent advances.
Tsao W, Jeong YH, Lin S, Ling J, Price DL, Chiang PM, Wong PC
Brain research 2012 Jun 26;1462:26-39
Brain research 2012 Jun 26;1462:26-39
Role of selected mutations in the Q/N rich region of TDP-43 in EGFP-12xQ/N-induced aggregate formation.
Budini M, Romano V, Avendaño-Vázquez SE, Bembich S, Buratti E, Baralle FE
Brain research 2012 Jun 26;1462:139-50
Brain research 2012 Jun 26;1462:139-50
Tunicamycin produces TDP-43 cytoplasmic inclusions in cultured brain organotypic slices.
Leggett C, McGehee DS, Mastrianni J, Yang W, Bai T, Brorson JR
Journal of the neurological sciences 2012 Jun 15;317(1-2):66-73
Journal of the neurological sciences 2012 Jun 15;317(1-2):66-73
HO-1 induction in motor cortex and intestinal dysfunction in TDP-43 A315T transgenic mice.
Guo Y, Wang Q, Zhang K, An T, Shi P, Li Z, Duan W, Li C
Brain research 2012 Jun 15;1460:88-95
Brain research 2012 Jun 15;1460:88-95
TDP-1/TDP-43 regulates stress signaling and age-dependent proteotoxicity in Caenorhabditis elegans.
Vaccaro A, Tauffenberger A, Ash PE, Carlomagno Y, Petrucelli L, Parker JA
PLoS genetics 2012 Jul;8(7):e1002806
PLoS genetics 2012 Jul;8(7):e1002806
Prevalence, laterality, and comorbidity of hippocampal sclerosis in an autopsy sample.
Zarow C, Weiner MW, Ellis WG, Chui HC
Brain and behavior 2012 Jul;2(4):435-42
Brain and behavior 2012 Jul;2(4):435-42
ALS-associated ataxin 2 polyQ expansions enhance stress-induced caspase 3 activation and increase TDP-43 pathological modifications.
Hart MP, Gitler AD
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Jul 4;32(27):9133-42
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Jul 4;32(27):9133-42
Clinical features of SCA36: a novel spinocerebellar ataxia with motor neuron involvement (Asidan).
Ikeda Y, Ohta Y, Kobayashi H, Okamoto M, Takamatsu K, Ota T, Manabe Y, Okamoto K, Koizumi A, Abe K
Neurology 2012 Jul 24;79(4):333-41
Neurology 2012 Jul 24;79(4):333-41
A role for SMN exon 7 splicing in the selective vulnerability of motor neurons in spinal muscular atrophy.
Ruggiu M, McGovern VL, Lotti F, Saieva L, Li DK, Kariya S, Monani UR, Burghes AH, Pellizzoni L
Molecular and cellular biology 2012 Jan;32(1):126-38
Molecular and cellular biology 2012 Jan;32(1):126-38
Absence of SOD1 leads to oxidative stress in peripheral nerve and causes a progressive distal motor axonopathy.
Fischer LR, Li Y, Asress SA, Jones DP, Glass JD
Experimental neurology 2012 Jan;233(1):163-71
Experimental neurology 2012 Jan;233(1):163-71
Transglutaminase 1 and its regulator tazarotene-induced gene 3 localize to neuronal tau inclusions in tauopathies.
Wilhelmus MM, de Jager M, Rozemuller AJ, Brevé J, Bol JG, Eckert RL, Drukarch B
The Journal of pathology 2012 Jan;226(1):132-42
The Journal of pathology 2012 Jan;226(1):132-42
Right temporal variant frontotemporal dementia with motor neuron disease.
Coon EA, Whitwell JL, Parisi JE, Dickson DW, Josephs KA
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2012 Jan;19(1):85-91
Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia 2012 Jan;19(1):85-91
Cognitive decline typical of frontotemporal lobar degeneration in transgenic mice expressing the 25-kDa C-terminal fragment of TDP-43.
Caccamo A, Majumder S, Oddo S
The American journal of pathology 2012 Jan;180(1):293-302
The American journal of pathology 2012 Jan;180(1):293-302
Supranuclear ophthalmoparesis and vacuolar degeneration of the cerebral white matter in amyotrophic lateral sclerosis: a clinicopathological study.
Takeda T, Uchihara T, Mochizuki Y, Ishihara A, Nakamura A, Sasaki S, Uchiyama S, Iwata M, Mizutani T
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Jan;13(1):74-83
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2012 Jan;13(1):74-83
National Institute on Aging-Alzheimer's Association guidelines for the neuropathologic assessment of Alzheimer's disease: a practical approach.
Montine TJ, Phelps CH, Beach TG, Bigio EH, Cairns NJ, Dickson DW, Duyckaerts C, Frosch MP, Masliah E, Mirra SS, Nelson PT, Schneider JA, Thal DR, Trojanowski JQ, Vinters HV, Hyman BT, National Institute on Aging, Alzheimer’s Association
Acta neuropathologica 2012 Jan;123(1):1-11
Acta neuropathologica 2012 Jan;123(1):1-11
A C9orf72 promoter repeat expansion in a Flanders-Belgian cohort with disorders of the frontotemporal lobar degeneration-amyotrophic lateral sclerosis spectrum: a gene identification study.
Gijselinck I, Van Langenhove T, van der Zee J, Sleegers K, Philtjens S, Kleinberger G, Janssens J, Bettens K, Van Cauwenberghe C, Pereson S, Engelborghs S, Sieben A, De Jonghe P, Vandenberghe R, Santens P, De Bleecker J, Maes G, Bäumer V, Dillen L, Joris G, Cuijt I, Corsmit E, Elinck E, Van Dongen J, Vermeulen S, Van den Broeck M, Vaerenberg C, Mattheijssens M, Peeters K, Robberecht W, Cras P, Martin JJ, De Deyn PP, Cruts M, Van Broeckhoven C
The Lancet. Neurology 2012 Jan;11(1):54-65
The Lancet. Neurology 2012 Jan;11(1):54-65
Epitope mapping of antibodies against TDP-43 and detection of protease-resistant fragments of pathological TDP-43 in amyotrophic lateral sclerosis and frontotemporal lobar degeneration.
Tsuji H, Nonaka T, Yamashita M, Masuda-Suzukake M, Kametani F, Akiyama H, Mann DM, Tamaoka A, Hasegawa M
Biochemical and biophysical research communications 2012 Jan 6;417(1):116-21
Biochemical and biophysical research communications 2012 Jan 6;417(1):116-21
A quantitative study of the neuropathology of 32 sporadic and familial cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Armstrong RA, Carter D, Cairns NJ
Neuropathology and applied neurobiology 2012 Feb;38(1):25-38
Neuropathology and applied neurobiology 2012 Feb;38(1):25-38
Alteration in cell cycle-related proteins in lymphoblasts from carriers of the c.709-1G>A PGRN mutation associated with FTLD-TDP dementia.
Alquezar C, Esteras N, Bartolomé F, Merino JJ, Alzualde A, López de Munain A, Martín-Requero Á
Neurobiology of aging 2012 Feb;33(2):429.e7-20
Neurobiology of aging 2012 Feb;33(2):429.e7-20
Unusual clinical presentation and neuropathology in two subjects with fused-in sarcoma (FUS) positive inclusions.
Hartikainen PH, Pikkarainen M, Hänninen T, Soininen H, Alafuzoff I
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Feb;32(1):60-8
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Feb;32(1):60-8
TDP-43 promotes microRNA biogenesis as a component of the Drosha and Dicer complexes.
Kawahara Y, Mieda-Sato A
Proceedings of the National Academy of Sciences of the United States of America 2012 Feb 28;109(9):3347-52
Proceedings of the National Academy of Sciences of the United States of America 2012 Feb 28;109(9):3347-52
Association of UBQLN1 mutation with Brown-Vialetto-Van Laere syndrome but not typical ALS.
González-Pérez P, Lu Y, Chian RJ, Sapp PC, Tanzi RE, Bertram L, McKenna-Yasek D, Gao FB, Brown RH Jr
Neurobiology of disease 2012 Dec;48(3):391-8
Neurobiology of disease 2012 Dec;48(3):391-8
Differential expression of TAR DNA-binding protein (TDP-43) in the central nervous system of horses afflicted with equine motor neuron disease (EMND): a preliminary study of a potential pathologic marker.
El-Assaad I, Di Bari JA, Yasuda K, Divers TJ, Summers BA, de Lahunta A, Mohammed H
Veterinary research communications 2012 Dec;36(4):221-6
Veterinary research communications 2012 Dec;36(4):221-6
Genetic association of CR1 with Alzheimer's disease: a tentative disease mechanism.
Hazrati LN, Van Cauwenberghe C, Brooks PL, Brouwers N, Ghani M, Sato C, Cruts M, Sleegers K, St George-Hyslop P, Van Broeckhoven C, Rogaeva E
Neurobiology of aging 2012 Dec;33(12):2949.e5-2949.e12
Neurobiology of aging 2012 Dec;33(12):2949.e5-2949.e12
Ultrastructure of ubiquitin-positive, TDP-43-negative neuronal inclusions in cerebral cortex of C9ORF72-linked frontotemporal lobar degeneration/amyotrophic lateral sclerosis.
Lin W, Dickson DW
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Dec;32(6):679-81
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Dec;32(6):679-81
Familial ALS with FUS P525L mutation: two Japanese sisters with multiple systems involvement.
Mochizuki Y, Isozaki E, Takao M, Hashimoto T, Shibuya M, Arai M, Hosokawa M, Kawata A, Oyanagi K, Mihara B, Mizutani T
Journal of the neurological sciences 2012 Dec 15;323(1-2):85-92
Journal of the neurological sciences 2012 Dec 15;323(1-2):85-92
Motor neuron-specific disruption of proteasomes, but not autophagy, replicates amyotrophic lateral sclerosis.
Tashiro Y, Urushitani M, Inoue H, Koike M, Uchiyama Y, Komatsu M, Tanaka K, Yamazaki M, Abe M, Misawa H, Sakimura K, Ito H, Takahashi R
The Journal of biological chemistry 2012 Dec 14;287(51):42984-94
The Journal of biological chemistry 2012 Dec 14;287(51):42984-94
Mitochondrial dysfunction in human TDP-43 transfected NSC34 cell lines and the protective effect of dimethoxy curcumin.
Lu J, Duan W, Guo Y, Jiang H, Li Z, Huang J, Hong K, Li C
Brain research bulletin 2012 Dec 1;89(5-6):185-90
Brain research bulletin 2012 Dec 1;89(5-6):185-90
Primary lateral sclerosis: upper-motor-predominant amyotrophic lateral sclerosis with frontotemporal lobar degeneration--immunohistochemical and biochemical analyses of TDP-43.
Kosaka T, Fu YJ, Shiga A, Ishidaira H, Tan CF, Tani T, Koike R, Onodera O, Nishizawa M, Kakita A, Takahashi H
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Aug;32(4):373-84
Neuropathology : official journal of the Japanese Society of Neuropathology 2012 Aug;32(4):373-84
Multiple system atrophy-parkinsonism with slow progression and prolonged survival: a diagnostic catch.
Petrovic IN, Ling H, Asi Y, Ahmed Z, Kukkle PL, Hazrati LN, Lang AE, Revesz T, Holton JL, Lees AJ
Movement disorders : official journal of the Movement Disorder Society 2012 Aug;27(9):1186-90
Movement disorders : official journal of the Movement Disorder Society 2012 Aug;27(9):1186-90
Reduced expression of BTBD10, an Akt activator, leads to motor neuron death.
Nawa M, Kage-Nakadai E, Aiso S, Okamoto K, Mitani S, Matsuoka M
Cell death and differentiation 2012 Aug;19(8):1398-407
Cell death and differentiation 2012 Aug;19(8):1398-407
TDP-43 pathology in a case of hereditary spastic paraplegia with a NIPA1/SPG6 mutation.
Martinez-Lage M, Molina-Porcel L, Falcone D, McCluskey L, Lee VM, Van Deerlin VM, Trojanowski JQ
Acta neuropathologica 2012 Aug;124(2):285-91
Acta neuropathologica 2012 Aug;124(2):285-91
Distinct TDP-43 pathology in ALS patients with ataxin 2 intermediate-length polyQ expansions.
Hart MP, Brettschneider J, Lee VM, Trojanowski JQ, Gitler AD
Acta neuropathologica 2012 Aug;124(2):221-30
Acta neuropathologica 2012 Aug;124(2):221-30
Asparaginyl endopeptidase cleaves TDP-43 in brain.
Herskowitz JH, Gozal YM, Duong DM, Dammer EB, Gearing M, Ye K, Lah JJ, Peng J, Levey AI, Seyfried NT
Proteomics 2012 Aug;12(15-16):2455-63
Proteomics 2012 Aug;12(15-16):2455-63
Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis.
Wu CH, Fallini C, Ticozzi N, Keagle PJ, Sapp PC, Piotrowska K, Lowe P, Koppers M, McKenna-Yasek D, Baron DM, Kost JE, Gonzalez-Perez P, Fox AD, Adams J, Taroni F, Tiloca C, Leclerc AL, Chafe SC, Mangroo D, Moore MJ, Zitzewitz JA, Xu ZS, van den Berg LH, Glass JD, Siciliano G, Cirulli ET, Goldstein DB, Salachas F, Meininger V, Rossoll W, Ratti A, Gellera C, Bosco DA, Bassell GJ, Silani V, Drory VE, Brown RH Jr, Landers JE
Nature 2012 Aug 23;488(7412):499-503
Nature 2012 Aug 23;488(7412):499-503
TMEM106B risk variant is implicated in the pathologic presentation of Alzheimer disease.
Rutherford NJ, Carrasquillo MM, Li M, Bisceglio G, Menke J, Josephs KA, Parisi JE, Petersen RC, Graff-Radford NR, Younkin SG, Dickson DW, Rademakers R
Neurology 2012 Aug 14;79(7):717-8
Neurology 2012 Aug 14;79(7):717-8
Targeted depletion of TDP-43 expression in the spinal cord motor neurons leads to the development of amyotrophic lateral sclerosis-like phenotypes in mice.
Wu LS, Cheng WC, Shen CK
The Journal of biological chemistry 2012 Aug 10;287(33):27335-44
The Journal of biological chemistry 2012 Aug 10;287(33):27335-44
Drug screening for ALS using patient-specific induced pluripotent stem cells.
Egawa N, Kitaoka S, Tsukita K, Naitoh M, Takahashi K, Yamamoto T, Adachi F, Kondo T, Okita K, Asaka I, Aoi T, Watanabe A, Yamada Y, Morizane A, Takahashi J, Ayaki T, Ito H, Yoshikawa K, Yamawaki S, Suzuki S, Watanabe D, Hioki H, Kaneko T, Makioka K, Okamoto K, Takuma H, Tamaoka A, Hasegawa K, Nonaka T, Hasegawa M, Kawata A, Yoshida M, Nakahata T, Takahashi R, Marchetto MC, Gage FH, Yamanaka S, Inoue H
Science translational medicine 2012 Aug 1;4(145):145ra104
Science translational medicine 2012 Aug 1;4(145):145ra104
Autoregulation of TDP-43 mRNA levels involves interplay between transcription, splicing, and alternative polyA site selection.
Avendaño-Vázquez SE, Dhir A, Bembich S, Buratti E, Proudfoot N, Baralle FE
Genes & development 2012 Aug 1;26(15):1679-84
Genes & development 2012 Aug 1;26(15):1679-84
Aquaporin-4 expression in distal myopathy with rimmed vacuoles.
Hoshi A, Yamamoto T, Kikuchi S, Soeda T, Shimizu K, Ugawa Y
BMC neurology 2012 Apr 27;12:22
BMC neurology 2012 Apr 27;12:22
Comparison of phosphorylated TDP-43-positive inclusions in oculomotor neurons in patients with non-ALS and ALS disorders.
Mizuno Y, Fujita Y, Takatama M, Okamoto K
Journal of the neurological sciences 2012 Apr 15;315(1-2):20-5
Journal of the neurological sciences 2012 Apr 15;315(1-2):20-5
Mutant induced pluripotent stem cell lines recapitulate aspects of TDP-43 proteinopathies and reveal cell-specific vulnerability.
Bilican B, Serio A, Barmada SJ, Nishimura AL, Sullivan GJ, Carrasco M, Phatnani HP, Puddifoot CA, Story D, Fletcher J, Park IH, Friedman BA, Daley GQ, Wyllie DJ, Hardingham GE, Wilmut I, Finkbeiner S, Maniatis T, Shaw CE, Chandran S
Proceedings of the National Academy of Sciences of the United States of America 2012 Apr 10;109(15):5803-8
Proceedings of the National Academy of Sciences of the United States of America 2012 Apr 10;109(15):5803-8
Prevention of motor neuron degeneration by novel iron chelators in SOD1(G93A) transgenic mice of amyotrophic lateral sclerosis.
Wang Q, Zhang X, Chen S, Zhang X, Zhang S, Youdium M, Le W
Neuro-degenerative diseases 2011;8(5):310-21
Neuro-degenerative diseases 2011;8(5):310-21
Cytoplasmic accumulation and aggregation of TDP-43 upon proteasome inhibition in cultured neurons.
van Eersel J, Ke YD, Gladbach A, Bi M, Götz J, Kril JJ, Ittner LM
PloS one 2011;6(7):e22850
PloS one 2011;6(7):e22850
Proteomic analysis of hippocampal dentate granule cells in frontotemporal lobar degeneration: application of laser capture technology.
Gozal YM, Dammer EB, Duong DM, Cheng D, Gearing M, Rees HD, Peng J, Lah JJ, Levey AI
Frontiers in neurology 2011;2:24
Frontiers in neurology 2011;2:24
A comprehensive assessment of the SOD1G93A low-copy transgenic mouse, which models human amyotrophic lateral sclerosis.
Acevedo-Arozena A, Kalmar B, Essa S, Ricketts T, Joyce P, Kent R, Rowe C, Parker A, Gray A, Hafezparast M, Thorpe JR, Greensmith L, Fisher EM
Disease models & mechanisms 2011 Sep;4(5):686-700
Disease models & mechanisms 2011 Sep;4(5):686-700
Temporoparietal atrophy: a marker of AD pathology independent of clinical diagnosis.
Whitwell JL, Jack CR Jr, Przybelski SA, Parisi JE, Senjem ML, Boeve BF, Knopman DS, Petersen RC, Dickson DW, Josephs KA
Neurobiology of aging 2011 Sep;32(9):1531-41
Neurobiology of aging 2011 Sep;32(9):1531-41
Pathological hallmarks of amyotrophic lateral sclerosis/frontotemporal lobar degeneration in transgenic mice produced with TDP-43 genomic fragments.
Swarup V, Phaneuf D, Bareil C, Robertson J, Rouleau GA, Kriz J, Julien JP
Brain : a journal of neurology 2011 Sep;134(Pt 9):2610-26
Brain : a journal of neurology 2011 Sep;134(Pt 9):2610-26
The clinical diagnosis of early-onset dementias: diagnostic accuracy and clinicopathological relationships.
Snowden JS, Thompson JC, Stopford CL, Richardson AM, Gerhard A, Neary D, Mann DM
Brain : a journal of neurology 2011 Sep;134(Pt 9):2478-92
Brain : a journal of neurology 2011 Sep;134(Pt 9):2478-92
Spinocerebellar ataxia type 2 (SCA2) is associated with TDP-43 pathology.
Toyoshima Y, Tanaka H, Shimohata M, Kimura K, Morita T, Kakita A, Takahashi H
Acta neuropathologica 2011 Sep;122(3):375-8
Acta neuropathologica 2011 Sep;122(3):375-8
Proportional neuronal loss between the primary motor and sensory cortex in amyotrophic lateral sclerosis.
Mochizuki Y, Mizutani T, Shimizu T, Kawata A
Neuroscience letters 2011 Sep 26;503(1):73-5
Neuroscience letters 2011 Sep 26;503(1):73-5
Immunohistochemical study of microscopic globular bodies of normal human brain.
Odagiri S, Mori F, Tanji K, Kuroda N, Wakabayashi K
Biomedical research (Tokyo, Japan) 2011 Oct;32(5):337-42
Biomedical research (Tokyo, Japan) 2011 Oct;32(5):337-42
An autopsy case of an aged patient with spinocerebellar ataxia type 2.
Ishida C, Komai K, Yonezawa K, Sakajiri K, Nitta E, Kawashima A, Yamada M
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Oct;31(5):510-8
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Oct;31(5):510-8
Diagnostic value of markers of muscle degeneration in sporadic inclusion body myositis.
Dubourg O, Wanschitz J, Maisonobe T, Béhin A, Allenbach Y, Herson S, Benveniste O
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2011 Oct;30(2):103-8
Acta myologica : myopathies and cardiomyopathies : official journal of the Mediterranean Society of Myology 2011 Oct;30(2):103-8
Astrocytes carrying the superoxide dismutase 1 (SOD1G93A) mutation induce wild-type motor neuron degeneration in vivo.
Papadeas ST, Kraig SE, O'Banion C, Lepore AC, Maragakis NJ
Proceedings of the National Academy of Sciences of the United States of America 2011 Oct 25;108(43):17803-8
Proceedings of the National Academy of Sciences of the United States of America 2011 Oct 25;108(43):17803-8
TDP-43 variants of frontotemporal lobar degeneration.
Bigio EH
Journal of molecular neuroscience : MN 2011 Nov;45(3):390-401
Journal of molecular neuroscience : MN 2011 Nov;45(3):390-401
Long-term observational study of sporadic inclusion body myositis.
Benveniste O, Guiguet M, Freebody J, Dubourg O, Squier W, Maisonobe T, Stojkovic T, Leite MI, Allenbach Y, Herson S, Brady S, Eymard B, Hilton-Jones D
Brain : a journal of neurology 2011 Nov;134(Pt 11):3176-84
Brain : a journal of neurology 2011 Nov;134(Pt 11):3176-84
An autopsy case of SOD1-related ALS with TDP-43 positive inclusions.
Okamoto Y, Ihara M, Urushitani M, Yamashita H, Kondo T, Tanigaki A, Oono M, Kawamata J, Ikemoto A, Kawamoto Y, Takahashi R, Ito H
Neurology 2011 Nov 29;77(22):1993-5
Neurology 2011 Nov 29;77(22):1993-5
Deregulation of TDP-43 in amyotrophic lateral sclerosis triggers nuclear factor κB-mediated pathogenic pathways.
Swarup V, Phaneuf D, Dupré N, Petri S, Strong M, Kriz J, Julien JP
The Journal of experimental medicine 2011 Nov 21;208(12):2429-47
The Journal of experimental medicine 2011 Nov 21;208(12):2429-47
Hippocampal sclerosis in advanced age: clinical and pathological features.
Nelson PT, Schmitt FA, Lin Y, Abner EL, Jicha GA, Patel E, Thomason PC, Neltner JH, Smith CD, Santacruz KS, Sonnen JA, Poon LW, Gearing M, Green RC, Woodard JL, Van Eldik LJ, Kryscio RJ
Brain : a journal of neurology 2011 May;134(Pt 5):1506-18
Brain : a journal of neurology 2011 May;134(Pt 5):1506-18
Granular expression of prolyl-peptidyl isomerase PIN1 is a constant and specific feature of Alzheimer's disease pathology and is independent of tau, Aβ and TDP-43 pathology.
Dakson A, Yokota O, Esiri M, Bigio EH, Horan M, Pendleton N, Richardson A, Neary D, Snowden JS, Robinson A, Davidson YS, Mann DM
Acta neuropathologica 2011 May;121(5):635-49
Acta neuropathologica 2011 May;121(5):635-49
Fronto-temporal lobar degeneration: neuropathology in 60 cases.
Seilhean D, Le Ber I, Sarazin M, Lacomblez L, Millecamps S, Salachas F, Pradat PF, Le Forestier N, LeGuern E, Dubois B, Meininger V, Brice A, Hauw JJ, Duyckaerts C
Journal of neural transmission (Vienna, Austria : 1996) 2011 May;118(5):753-64
Journal of neural transmission (Vienna, Austria : 1996) 2011 May;118(5):753-64
TDP-43 neurotoxicity and protein aggregation modulated by heat shock factor and insulin/IGF-1 signaling.
Zhang T, Mullane PC, Periz G, Wang J
Human molecular genetics 2011 May 15;20(10):1952-65
Human molecular genetics 2011 May 15;20(10):1952-65
A family with Parkinsonism, essential tremor, restless legs syndrome, and depression.
Puschmann A, Pfeiffer RF, Stoessl AJ, Kuriakose R, Lash JL, Searcy JA, Strongosky AJ, Vilariño-Güell C, Farrer MJ, Ross OA, Dickson DW, Wszolek ZK
Neurology 2011 May 10;76(19):1623-30
Neurology 2011 May 10;76(19):1623-30
FTLD-TDP with motor neuron disease, visuospatial impairment and a progressive supranuclear palsy-like syndrome: broadening the clinical phenotype of TDP-43 proteinopathies. A report of three cases.
Rusina R, Kovacs GG, Fiala J, Hort J, Ridzoň P, Holmerová I, Ströbel T, Matěj R
BMC neurology 2011 May 10;11:50
BMC neurology 2011 May 10;11:50
A novel double mutation in FUS gene causing sporadic ALS.
Robertson J, Bilbao J, Zinman L, Hazrati LN, Tokuhiro S, Sato C, Moreno D, Strome R, Mackenzie IR, Rogaeva E
Neurobiology of aging 2011 Mar;32(3):553.e27-30
Neurobiology of aging 2011 Mar;32(3):553.e27-30
TDP-43 is directed to stress granules by sorbitol, a novel physiological osmotic and oxidative stressor.
Dewey CM, Cenik B, Sephton CF, Dries DR, Mayer P 3rd, Good SK, Johnson BA, Herz J, Yu G
Molecular and cellular biology 2011 Mar;31(5):1098-108
Molecular and cellular biology 2011 Mar;31(5):1098-108
New neuropathological findings in Unverricht-Lundborg disease: neuronal intranuclear and cytoplasmic inclusions.
Cohen NR, Hammans SR, Macpherson J, Nicoll JA
Acta neuropathologica 2011 Mar;121(3):421-7
Acta neuropathologica 2011 Mar;121(3):421-7
Pathological correlates of frontotemporal lobar degeneration in the elderly.
Baborie A, Griffiths TD, Jaros E, McKeith IG, Burn DJ, Richardson A, Ferrari R, Moreno J, Momeni P, Duplessis D, Pal P, Rollinson S, Pickering-Brown S, Thompson JC, Neary D, Snowden JS, Perry R, Mann DM
Acta neuropathologica 2011 Mar;121(3):365-71
Acta neuropathologica 2011 Mar;121(3):365-71
Elevated CSF TDP-43 levels in amyotrophic lateral sclerosis: specificity, sensitivity, and a possible prognostic value.
Noto Y, Shibuya K, Sato Y, Kanai K, Misawa S, Sawai S, Mori M, Uchiyama T, Isose S, Nasu S, Sekiguchi Y, Fujimaki Y, Kasai T, Tokuda T, Nakagawa M, Kuwabara S
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2011 Mar;12(2):140-3
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2011 Mar;12(2):140-3
TDP-43 potentiates alpha-synuclein toxicity to dopaminergic neurons in transgenic mice.
Tian T, Huang C, Tong J, Yang M, Zhou H, Xia XG
International journal of biological sciences 2011 Mar 4;7(2):234-43
International journal of biological sciences 2011 Mar 4;7(2):234-43
TDP-43 pathology occurs infrequently in multiple system atrophy.
Geser F, Malunda JA, Hurtig HI, Duda JE, Wenning GK, Gilman S, Low PA, Lee VM, Trojanowski JQ
Neuropathology and applied neurobiology 2011 Jun;37(4):358-65
Neuropathology and applied neurobiology 2011 Jun;37(4):358-65
Semantic dementia with lower motor neuron disease showing FTLD-TDP type 3 pathology (sensu Mackenzie).
Östberg P, Bogdanović N
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Jun;31(3):271-9
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Jun;31(3):271-9
Ubiquitinated, p62 immunopositive cerebellar cortical neuronal inclusions are evident across the spectrum of TDP-43 proteinopathies but are only rarely additionally immunopositive for phosphorylation-dependent TDP-43.
King A, Maekawa S, Bodi I, Troakes C, Al-Sarraj S
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Jun;31(3):239-49
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Jun;31(3):239-49
Catastrophic hyperkalaemia following administration of suxamethonium chloride to a patient with undiagnosed amyotrophic lateral sclerosis.
Turner MR, Lawrence H, Arnold I, Ansorge O, Talbot K
Clinical medicine (London, England) 2011 Jun;11(3):292-3
Clinical medicine (London, England) 2011 Jun;11(3):292-3
An ALS-associated mutation affecting TDP-43 enhances protein aggregation, fibril formation and neurotoxicity.
Guo W, Chen Y, Zhou X, Kar A, Ray P, Chen X, Rao EJ, Yang M, Ye H, Zhu L, Liu J, Xu M, Yang Y, Wang C, Zhang D, Bigio EH, Mesulam M, Shen Y, Xu Q, Fushimi K, Wu JY
Nature structural & molecular biology 2011 Jun 12;18(7):822-30
Nature structural & molecular biology 2011 Jun 12;18(7):822-30
RNA targets of TDP-43 identified by UV-CLIP are deregulated in ALS.
Xiao S, Sanelli T, Dib S, Sheps D, Findlater J, Bilbao J, Keith J, Zinman L, Rogaeva E, Robertson J
Molecular and cellular neurosciences 2011 Jul;47(3):167-80
Molecular and cellular neurosciences 2011 Jul;47(3):167-80
The most common type of FTLD-FUS (aFTLD-U) is associated with a distinct clinical form of frontotemporal dementia but is not related to mutations in the FUS gene.
Snowden JS, Hu Q, Rollinson S, Halliwell N, Robinson A, Davidson YS, Momeni P, Baborie A, Griffiths TD, Jaros E, Perry RH, Richardson A, Pickering-Brown SM, Neary D, Mann DM
Acta neuropathologica 2011 Jul;122(1):99-110
Acta neuropathologica 2011 Jul;122(1):99-110
A Drosophila model of FUS-related neurodegeneration reveals genetic interaction between FUS and TDP-43.
Lanson NA Jr, Maltare A, King H, Smith R, Kim JH, Taylor JP, Lloyd TE, Pandey UB
Human molecular genetics 2011 Jul 1;20(13):2510-23
Human molecular genetics 2011 Jul 1;20(13):2510-23
Nuclear transport impairment of amyotrophic lateral sclerosis-linked mutations in FUS/TLS.
Ito D, Seki M, Tsunoda Y, Uchiyama H, Suzuki N
Annals of neurology 2011 Jan;69(1):152-62
Annals of neurology 2011 Jan;69(1):152-62
Regulation of TDP-43 aggregation by phosphorylation and p62/SQSTM1.
Brady OA, Meng P, Zheng Y, Mao Y, Hu F
Journal of neurochemistry 2011 Jan;116(2):248-59
Journal of neurochemistry 2011 Jan;116(2):248-59
TDP-43 regulates its mRNA levels through a negative feedback loop.
Ayala YM, De Conti L, Avendaño-Vázquez SE, Dhir A, Romano M, D'Ambrogio A, Tollervey J, Ule J, Baralle M, Buratti E, Baralle FE
The EMBO journal 2011 Jan 19;30(2):277-88
The EMBO journal 2011 Jan 19;30(2):277-88
FALS with Gly72Ser mutation in SOD1 gene: report of a family including the first autopsy case.
Kobayashi Z, Tsuchiya K, Kubodera T, Shibata N, Arai T, Miura H, Ishikawa C, Kondo H, Ishizu H, Akiyama H, Mizusawa H
Journal of the neurological sciences 2011 Jan 15;300(1-2):9-13
Journal of the neurological sciences 2011 Jan 15;300(1-2):9-13
Identification of neuronal RNA targets of TDP-43-containing ribonucleoprotein complexes.
Sephton CF, Cenik C, Kucukural A, Dammer EB, Cenik B, Han Y, Dewey CM, Roth FP, Herz J, Peng J, Moore MJ, Yu G
The Journal of biological chemistry 2011 Jan 14;286(2):1204-15
The Journal of biological chemistry 2011 Jan 14;286(2):1204-15
Biochemical, neuropathological, and neuroimaging characteristics of early-onset Alzheimer's disease due to a novel PSEN1 mutation.
Ringman JM, Gylys KH, Medina LD, Fox M, Kepe V, Flores DL, Apostolova LG, Barrio JR, Small G, Silverman DH, Siu E, Cederbaum S, Hecimovic S, Malnar M, Chakraverty S, Goate AM, Bird TD, Leverenz JB
Neuroscience letters 2011 Jan 10;487(3):287-92
Neuroscience letters 2011 Jan 10;487(3):287-92
Clinical, neuroimaging and neuropathological features of a new chromosome 9p-linked FTD-ALS family.
Boxer AL, Mackenzie IR, Boeve BF, Baker M, Seeley WW, Crook R, Feldman H, Hsiung GY, Rutherford N, Laluz V, Whitwell J, Foti D, McDade E, Molano J, Karydas A, Wojtas A, Goldman J, Mirsky J, Sengdy P, Dearmond S, Miller BL, Rademakers R
Journal of neurology, neurosurgery, and psychiatry 2011 Feb;82(2):196-203
Journal of neurology, neurosurgery, and psychiatry 2011 Feb;82(2):196-203
Overview and recent advances in neuropathology. Part 2: Neurodegeneration.
Masters CL, Kril JJ, Halliday GM, Pamphlett R, Collins S, Hill AF, McLean C
Pathology 2011 Feb;43(2):93-102
Pathology 2011 Feb;43(2):93-102
Both cytoplasmic and nuclear accumulations of the protein are neurotoxic in Drosophila models of TDP-43 proteinopathies.
Miguel L, Frébourg T, Campion D, Lecourtois M
Neurobiology of disease 2011 Feb;41(2):398-406
Neurobiology of disease 2011 Feb;41(2):398-406
A morphometric study of the spatial patterns of TDP-43 immunoreactive neuronal inclusions in frontotemporal lobar degeneration (FTLD) with progranulin (GRN) mutation.
Armstrong RA, Cairns NJ
Histology and histopathology 2011 Feb;26(2):185-90
Histology and histopathology 2011 Feb;26(2):185-90
Dysregulation of the ALS-associated gene TDP-43 leads to neuronal death and degeneration in mice.
Igaz LM, Kwong LK, Lee EB, Chen-Plotkin A, Swanson E, Unger T, Malunda J, Xu Y, Winton MJ, Trojanowski JQ, Lee VM
The Journal of clinical investigation 2011 Feb;121(2):726-38
The Journal of clinical investigation 2011 Feb;121(2):726-38
Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation.
Camdessanché JP, Belzil VV, Jousserand G, Rouleau GA, Créac'h C, Convers P, Antoine JC
Orphanet journal of rare diseases 2011 Feb 5;6:4
Orphanet journal of rare diseases 2011 Feb 5;6:4
Inorganic mercury within motor neurons does not cause the TDP-43 changes seen in sporadic ALS.
Pamphlett R, Kum Jew S
Toxicology letters 2011 Feb 25;201(1):58-61
Toxicology letters 2011 Feb 25;201(1):58-61
Clinicopathological study of diffuse neurofibrillary tangles with calcification. With special reference to TDP-43 proteinopathy and alpha-synucleinopathy.
Habuchi C, Iritani S, Sekiguchi H, Torii Y, Ishihara R, Arai T, Hasegawa M, Tsuchiya K, Akiyama H, Shibayama H, Ozaki N
Journal of the neurological sciences 2011 Feb 15;301(1-2):77-85
Journal of the neurological sciences 2011 Feb 15;301(1-2):77-85
The ALS/PDC syndrome of Guam: potential biomarkers for an enigmatic disorder.
McGeer PL, Steele JC
Progress in neurobiology 2011 Dec;95(4):663-9
Progress in neurobiology 2011 Dec;95(4):663-9
Abnormal TDP-43 and FUS proteins in muscles of sporadic IBM: similarities in a TARDBP-linked ALS patient.
Hernandez Lain A, Millecamps S, Dubourg O, Salachas F, Bruneteau G, Lacomblez L, LeGuern E, Seilhean D, Duyckaerts C, Meininger V, Mallet J, Pradat PF
Journal of neurology, neurosurgery, and psychiatry 2011 Dec;82(12):1414-6
Journal of neurology, neurosurgery, and psychiatry 2011 Dec;82(12):1414-6
Molecular properties of TAR DNA binding protein-43 fragments are dependent upon its cleavage site.
Furukawa Y, Kaneko K, Nukina N
Biochimica et biophysica acta 2011 Dec;1812(12):1577-83
Biochimica et biophysica acta 2011 Dec;1812(12):1577-83
Acute and chronically increased immunoreactivity to phosphorylation-independent but not pathological TDP-43 after a single traumatic brain injury in humans.
Johnson VE, Stewart W, Trojanowski JQ, Smith DH
Acta neuropathologica 2011 Dec;122(6):715-26
Acta neuropathologica 2011 Dec;122(6):715-26
TDP-43 pathological changes in early onset familial and sporadic Alzheimer's disease, late onset Alzheimer's disease and Down's syndrome: association with age, hippocampal sclerosis and clinical phenotype.
Davidson YS, Raby S, Foulds PG, Robinson A, Thompson JC, Sikkink S, Yusuf I, Amin H, DuPlessis D, Troakes C, Al-Sarraj S, Sloan C, Esiri MM, Prasher VP, Allsop D, Neary D, Pickering-Brown SM, Snowden JS, Mann DM
Acta neuropathologica 2011 Dec;122(6):703-13
Acta neuropathologica 2011 Dec;122(6):703-13
Diacetylbis(N(4)-methylthiosemicarbazonato) copper(II) (CuII(atsm)) protects against peroxynitrite-induced nitrosative damage and prolongs survival in amyotrophic lateral sclerosis mouse model.
Soon CPW, Donnelly PS, Turner BJ, Hung LW, Crouch PJ, Sherratt NA, Tan JL, Lim NK, Lam L, Bica L, Lim S, Hickey JL, Morizzi J, Powell A, Finkelstein DI, Culvenor JG, Masters CL, Duce J, White AR, Barnham KJ, Li QX
The Journal of biological chemistry 2011 Dec 23;286(51):44035-44044
The Journal of biological chemistry 2011 Dec 23;286(51):44035-44044
FUS and TARDBP but not SOD1 interact in genetic models of amyotrophic lateral sclerosis.
Kabashi E, Bercier V, Lissouba A, Liao M, Brustein E, Rouleau GA, Drapeau P
PLoS genetics 2011 Aug;7(8):e1002214
PLoS genetics 2011 Aug;7(8):e1002214
Differential involvement of optineurin in amyotrophic lateral sclerosis with or without SOD1 mutations.
Deng HX, Bigio EH, Zhai H, Fecto F, Ajroud K, Shi Y, Yan J, Mishra M, Ajroud-Driss S, Heller S, Sufit R, Siddique N, Mugnaini E, Siddique T
Archives of neurology 2011 Aug;68(8):1057-61
Archives of neurology 2011 Aug;68(8):1057-61
Spatial patterns of TDP-43 neuronal cytoplasmic inclusions (NCI) in fifteen cases of frontotemporal lobar degeneration with TDP-43 proteinopathy (FTLD-TDP).
Armstrong RA, Cairns NJ
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2011 Aug;32(4):653-9
Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2011 Aug;32(4):653-9
Enhancement of native and phosphorylated TDP-43 immunoreactivity by proteinase K treatment following autoclave heating.
Mori F, Tanji K, Kakita A, Takahashi H, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Aug;31(4):401-4
Neuropathology : official journal of the Japanese Society of Neuropathology 2011 Aug;31(4):401-4
Clinicopathologic study on an ALS family with a heterozygous E478G optineurin mutation.
Ito H, Nakamura M, Komure O, Ayaki T, Wate R, Maruyama H, Nakamura Y, Fujita K, Kaneko S, Okamoto Y, Ihara M, Konishi T, Ogasawara K, Hirano A, Kusaka H, Kaji R, Takahashi R, Kawakami H
Acta neuropathologica 2011 Aug;122(2):223-9
Acta neuropathologica 2011 Aug;122(2):223-9
A peculiar constellation of tau pathology defines a subset of dementia in the elderly.
Kovacs GG, Molnár K, László L, Ströbel T, Botond G, Hönigschnabl S, Reiner-Concin A, Palkovits M, Fischer P, Budka H
Acta neuropathologica 2011 Aug;122(2):205-22
Acta neuropathologica 2011 Aug;122(2):205-22
C-Jun N-terminal kinase controls TDP-43 accumulation in stress granules induced by oxidative stress.
Meyerowitz J, Parker SJ, Vella LJ, Ng DCh, Price KA, Liddell JR, Caragounis A, Li QX, Masters CL, Nonaka T, Hasegawa M, Bogoyevitch MA, Kanninen KM, Crouch PJ, White AR
Molecular neurodegeneration 2011 Aug 8;6:57
Molecular neurodegeneration 2011 Aug 8;6:57
TDP-43 knockdown impairs neurite outgrowth dependent on its target histone deacetylase 6.
Fiesel FC, Schurr C, Weber SS, Kahle PJ
Molecular neurodegeneration 2011 Aug 30;6:64
Molecular neurodegeneration 2011 Aug 30;6:64
Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia.
Deng HX, Chen W, Hong ST, Boycott KM, Gorrie GH, Siddique N, Yang Y, Fecto F, Shi Y, Zhai H, Jiang H, Hirano M, Rampersaud E, Jansen GH, Donkervoort S, Bigio EH, Brooks BR, Ajroud K, Sufit RL, Haines JL, Mugnaini E, Pericak-Vance MA, Siddique T
Nature 2011 Aug 21;477(7363):211-5
Nature 2011 Aug 21;477(7363):211-5
Familial prion disease with Alzheimer disease-like tau pathology and clinical phenotype.
Jayadev S, Nochlin D, Poorkaj P, Steinbart EJ, Mastrianni JA, Montine TJ, Ghetti B, Schellenberg GD, Bird TD, Leverenz JB
Annals of neurology 2011 Apr;69(4):712-20
Annals of neurology 2011 Apr;69(4):712-20
In sporadic inclusion body myositis muscle fibres TDP-43-positive inclusions are less frequent and robust than p62 inclusions, and are not associated with paired helical filaments.
D'Agostino C, Nogalska A, Engel WK, Askanas V
Neuropathology and applied neurobiology 2011 Apr;37(3):315-20
Neuropathology and applied neurobiology 2011 Apr;37(3):315-20
Familial frontotemporal dementia with amyotrophic lateral sclerosis and a shared haplotype on chromosome 9p.
Pearson JP, Williams NM, Majounie E, Waite A, Stott J, Newsway V, Murray A, Hernandez D, Guerreiro R, Singleton AB, Neal J, Morris HR
Journal of neurology 2011 Apr;258(4):647-55
Journal of neurology 2011 Apr;258(4):647-55
Long pre-mRNA depletion and RNA missplicing contribute to neuronal vulnerability from loss of TDP-43.
Polymenidou M, Lagier-Tourenne C, Hutt KR, Huelga SC, Moran J, Liang TY, Ling SC, Sun E, Wancewicz E, Mazur C, Kordasiewicz H, Sedaghat Y, Donohue JP, Shiue L, Bennett CF, Yeo GW, Cleveland DW
Nature neuroscience 2011 Apr;14(4):459-68
Nature neuroscience 2011 Apr;14(4):459-68
Characterizing the RNA targets and position-dependent splicing regulation by TDP-43.
Tollervey JR, Curk T, Rogelj B, Briese M, Cereda M, Kayikci M, König J, Hortobágyi T, Nishimura AL, Zupunski V, Patani R, Chandran S, Rot G, Zupan B, Shaw CE, Ule J
Nature neuroscience 2011 Apr;14(4):452-8
Nature neuroscience 2011 Apr;14(4):452-8
Novel histopathologic findings in molecularly-confirmed pantothenate kinase-associated neurodegeneration.
Kruer MC, Hiken M, Gregory A, Malandrini A, Clark D, Hogarth P, Grafe M, Hayflick SJ, Woltjer RL
Brain : a journal of neurology 2011 Apr;134(Pt 4):947-58
Brain : a journal of neurology 2011 Apr;134(Pt 4):947-58
TDP-43 pathology may occur in the BRI2 gene-related dementias.
Lashley T, Holton JL, Revesz T
Acta neuropathologica 2011 Apr;121(4):559-60
Acta neuropathologica 2011 Apr;121(4):559-60
Motor neuron disease clinically limited to the lower motor neuron is a diffuse TDP-43 proteinopathy.
Geser F, Stein B, Partain M, Elman LB, McCluskey LF, Xie SX, Van Deerlin VM, Kwong LK, Lee VM, Trojanowski JQ
Acta neuropathologica 2011 Apr;121(4):509-17
Acta neuropathologica 2011 Apr;121(4):509-17
Novel progranulin mutation detected in 2 patients with FTLD.
Skoglund L, Matsui T, Freeman SH, Wallin A, Blom ES, Frosch MP, Growdon JH, Hyman BT, Lannfelt L, Ingelsson M, Glaser A
Alzheimer disease and associated disorders 2011 Apr-Jun;25(2):173-8
Alzheimer disease and associated disorders 2011 Apr-Jun;25(2):173-8
ApoE E4 is a susceptibility factor in amnestic but not aphasic dementias.
Rogalski EJ, Rademaker A, Harrison TM, Helenowski I, Johnson N, Bigio E, Mishra M, Weintraub S, Mesulam MM
Alzheimer disease and associated disorders 2011 Apr-Jun;25(2):159-63
Alzheimer disease and associated disorders 2011 Apr-Jun;25(2):159-63
β-amyloid triggers ALS-associated TDP-43 pathology in AD models.
Herman AM, Khandelwal PJ, Stanczyk BB, Rebeck GW, Moussa CE
Brain research 2011 Apr 22;1386:191-9
Brain research 2011 Apr 22;1386:191-9
TDP-43-induced death is associated with altered regulation of BIM and Bcl-xL and attenuated by caspase-mediated TDP-43 cleavage.
Suzuki H, Lee K, Matsuoka M
The Journal of biological chemistry 2011 Apr 15;286(15):13171-83
The Journal of biological chemistry 2011 Apr 15;286(15):13171-83
TAR DNA-binding protein 43 (TDP-43) regulates stress granule dynamics via differential regulation of G3BP and TIA-1.
McDonald KK, Aulas A, Destroismaisons L, Pickles S, Beleac E, Camu W, Rouleau GA, Vande Velde C
Human molecular genetics 2011 Apr 1;20(7):1400-10
Human molecular genetics 2011 Apr 1;20(7):1400-10
TDP43-positive intraneuronal inclusions in a patient with motor neuron disease and Parkinson's disease.
Chanson JB, Echaniz-Laguna A, Vogel T, Mohr M, Benoilid A, Kaltenbach G, Kiesmann M
Neuro-degenerative diseases 2010;7(4):260-4
Neuro-degenerative diseases 2010;7(4):260-4
TDP-43 M337V mutation in familial amyotrophic lateral sclerosis in Japan.
Tamaoka A, Arai M, Itokawa M, Arai T, Hasegawa M, Tsuchiya K, Takuma H, Tsuji H, Ishii A, Watanabe M, Takahashi Y, Goto J, Tsuji S, Akiyama H
Internal medicine (Tokyo, Japan) 2010;49(4):331-4
Internal medicine (Tokyo, Japan) 2010;49(4):331-4
Intra-familial clinical heterogeneity due to FTLD-U with TDP-43 proteinopathy caused by a novel deletion in progranulin gene (PGRN).
Gabryelewicz T, Masellis M, Berdynski M, Bilbao JM, Rogaeva E, St George-Hyslop P, Barczak A, Czyzewski K, Barcikowska M, Wszolek Z, Black SE, Zekanowski C
Journal of Alzheimer's disease : JAD 2010;22(4):1123-33
Journal of Alzheimer's disease : JAD 2010;22(4):1123-33
Increased neuronal Rab5 immunoreactive endosomes do not colocalize with TDP-43 in motor neuron disease.
Matej R, Botond G, László L, Kopitar-Jerala N, Rusina R, Budka H, Kovacs GG
Experimental neurology 2010 Sep;225(1):133-9
Experimental neurology 2010 Sep;225(1):133-9
Altered distributions of Gemini of coiled bodies and mitochondria in motor neurons of TDP-43 transgenic mice.
Shan X, Chiang PM, Price DL, Wong PC
Proceedings of the National Academy of Sciences of the United States of America 2010 Sep 14;107(37):16325-30
Proceedings of the National Academy of Sciences of the United States of America 2010 Sep 14;107(37):16325-30
Deletion of TDP-43 down-regulates Tbc1d1, a gene linked to obesity, and alters body fat metabolism.
Chiang PM, Ling J, Jeong YH, Price DL, Aja SM, Wong PC
Proceedings of the National Academy of Sciences of the United States of America 2010 Sep 14;107(37):16320-4
Proceedings of the National Academy of Sciences of the United States of America 2010 Sep 14;107(37):16320-4
Lack of shunt response in suspected idiopathic normal pressure hydrocephalus with Alzheimer disease pathology.
Hamilton R, Patel S, Lee EB, Jackson EM, Lopinto J, Arnold SE, Clark CM, Basil A, Shaw LM, Xie SX, Grady MS, Trojanowski JQ
Annals of neurology 2010 Oct;68(4):535-40
Annals of neurology 2010 Oct;68(4):535-40
Pathological 43-kDa transactivation response DNA-binding protein in older adults with and without severe mental illness.
Geser F, Robinson JL, Malunda JA, Xie SX, Clark CM, Kwong LK, Moberg PJ, Moore EM, Van Deerlin VM, Lee VM, Arnold SE, Trojanowski JQ
Archives of neurology 2010 Oct;67(10):1238-50
Archives of neurology 2010 Oct;67(10):1238-50
Age-related motor neuron degeneration in DNA repair-deficient Ercc1 mice.
de Waard MC, van der Pluijm I, Zuiderveen Borgesius N, Comley LH, Haasdijk ED, Rijksen Y, Ridwan Y, Zondag G, Hoeijmakers JH, Elgersma Y, Gillingwater TH, Jaarsma D
Acta neuropathologica 2010 Oct;120(4):461-75
Acta neuropathologica 2010 Oct;120(4):461-75
Involvement of Onuf's nucleus in Machado-Joseph disease: a morphometric and immunohistochemical study.
Shimizu H, Yamada M, Toyoshima Y, Ikeuchi T, Onodera O, Takahashi H
Acta neuropathologica 2010 Oct;120(4):439-48
Acta neuropathologica 2010 Oct;120(4):439-48
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.
Kim SH, Shanware NP, Bowler MJ, Tibbetts RS
The Journal of biological chemistry 2010 Oct 29;285(44):34097-105
The Journal of biological chemistry 2010 Oct 29;285(44):34097-105
Progranulin is neurotrophic in vivo and protects against a mutant TDP-43 induced axonopathy.
Laird AS, Van Hoecke A, De Muynck L, Timmers M, Van den Bosch L, Van Damme P, Robberecht W
PloS one 2010 Oct 13;5(10):e13368
PloS one 2010 Oct 13;5(10):e13368
Tar DNA binding protein-43 (TDP-43) associates with stress granules: analysis of cultured cells and pathological brain tissue.
Liu-Yesucevitz L, Bilgutay A, Zhang YJ, Vanderweyde T, Citro A, Mehta T, Zaarur N, McKee A, Bowser R, Sherman M, Petrucelli L, Wolozin B
PloS one 2010 Oct 11;5(10):e13250
PloS one 2010 Oct 11;5(10):e13250
Sigma nonopioid intracellular receptor 1 mutations cause frontotemporal lobar degeneration-motor neuron disease.
Luty AA, Kwok JB, Dobson-Stone C, Loy CT, Coupland KG, Karlström H, Sobow T, Tchorzewska J, Maruszak A, Barcikowska M, Panegyres PK, Zekanowski C, Brooks WS, Williams KL, Blair IP, Mather KA, Sachdev PS, Halliday GM, Schofield PR
Annals of neurology 2010 Nov;68(5):639-49
Annals of neurology 2010 Nov;68(5):639-49
Extensive FUS-immunoreactive pathology in juvenile amyotrophic lateral sclerosis with basophilic inclusions.
Huang EJ, Zhang J, Geser F, Trojanowski JQ, Strober JB, Dickson DW, Brown RH Jr, Shapiro BE, Lomen-Hoerth C
Brain pathology (Zurich, Switzerland) 2010 Nov;20(6):1069-76
Brain pathology (Zurich, Switzerland) 2010 Nov;20(6):1069-76
Increased caspase activation and decreased TDP-43 solubility in progranulin knockout cortical cultures.
Kleinberger G, Wils H, Ponsaerts P, Joris G, Timmermans JP, Van Broeckhoven C, Kumar-Singh S
Journal of neurochemistry 2010 Nov;115(3):735-47
Journal of neurochemistry 2010 Nov;115(3):735-47
Age-dependent changes in TDP-43 levels in a mouse model of Alzheimer disease are linked to Aβ oligomers accumulation.
Caccamo A, Magrí A, Oddo S
Molecular neurodegeneration 2010 Nov 11;5:51
Molecular neurodegeneration 2010 Nov 11;5:51
Efficient conditional and promoter-specific in vivo expression of cDNAs of choice by taking advantage of recombinase-mediated cassette exchange using FlEx gene traps.
Schebelle L, Wolf C, Stribl C, Javaheri T, Schnütgen F, Ettinger A, Ivics Z, Hansen J, Ruiz P, von Melchner H, Wurst W, Floss T
Nucleic acids research 2010 May;38(9):e106
Nucleic acids research 2010 May;38(9):e106
TDP-43 dimerizes in human cells in culture.
Shiina Y, Arima K, Tabunoki H, Satoh J
Cellular and molecular neurobiology 2010 May;30(4):641-52
Cellular and molecular neurobiology 2010 May;30(4):641-52
FUS-immunoreactive intranuclear inclusions in neurodegenerative disease.
Woulfe J, Gray DA, Mackenzie IR
Brain pathology (Zurich, Switzerland) 2010 May;20(3):589-97
Brain pathology (Zurich, Switzerland) 2010 May;20(3):589-97
A mutation affecting the sodium/proton exchanger, SLC9A6, causes mental retardation with tau deposition.
Garbern JY, Neumann M, Trojanowski JQ, Lee VM, Feldman G, Norris JW, Friez MJ, Schwartz CE, Stevenson R, Sima AA
Brain : a journal of neurology 2010 May;133(Pt 5):1391-402
Brain : a journal of neurology 2010 May;133(Pt 5):1391-402
TDP-43 and ubiquitinated cytoplasmic aggregates in sporadic ALS are low frequency and widely distributed in the lower motor neuron columns independent of disease spread.
Bodansky A, Kim JM, Tempest L, Velagapudi A, Libby R, Ravits J
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2010 May 3;11(3):321-7
Amyotrophic lateral sclerosis : official publication of the World Federation of Neurology Research Group on Motor Neuron Diseases 2010 May 3;11(3):321-7
Mutant superoxide dismutase 1 overexpression in NSC-34 cells: effect of trehalose on aggregation, TDP-43 localization and levels of co-expressed glycoproteins.
Gomes C, Escrevente C, Costa J
Neuroscience letters 2010 May 21;475(3):145-9
Neuroscience letters 2010 May 21;475(3):145-9
Amyotrophic lateral sclerosis mutant vesicle-associated membrane protein-associated protein-B transgenic mice develop TAR-DNA-binding protein-43 pathology.
Tudor EL, Galtrey CM, Perkinton MS, Lau KF, De Vos KJ, Mitchell JC, Ackerley S, Hortobágyi T, Vámos E, Leigh PN, Klasen C, McLoughlin DM, Shaw CE, Miller CC
Neuroscience 2010 May 19;167(3):774-85
Neuroscience 2010 May 19;167(3):774-85
Mutations of optineurin in amyotrophic lateral sclerosis.
Maruyama H, Morino H, Ito H, Izumi Y, Kato H, Watanabe Y, Kinoshita Y, Kamada M, Nodera H, Suzuki H, Komure O, Matsuura S, Kobatake K, Morimoto N, Abe K, Suzuki N, Aoki M, Kawata A, Hirai T, Kato T, Ogasawara K, Hirano A, Takumi T, Kusaka H, Hagiwara K, Kaji R, Kawakami H
Nature 2010 May 13;465(7295):223-6
Nature 2010 May 13;465(7295):223-6
Transgenic mice expressing mutant forms VCP/p97 recapitulate the full spectrum of IBMPFD including degeneration in muscle, brain and bone.
Custer SK, Neumann M, Lu H, Wright AC, Taylor JP
Human molecular genetics 2010 May 1;19(9):1741-55
Human molecular genetics 2010 May 1;19(9):1741-55
Synergistic effect between proteasome and autophagosome in the clearance of polyubiquitinated TDP-43.
Urushitani M, Sato T, Bamba H, Hisa Y, Tooyama I
Journal of neuroscience research 2010 Mar;88(4):784-97
Journal of neuroscience research 2010 Mar;88(4):784-97
TDP-43 redistribution is an early event in sporadic amyotrophic lateral sclerosis.
Giordana MT, Piccinini M, Grifoni S, De Marco G, Vercellino M, Magistrello M, Pellerino A, Buccinnà B, Lupino E, Rinaudo MT
Brain pathology (Zurich, Switzerland) 2010 Mar;20(2):351-60
Brain pathology (Zurich, Switzerland) 2010 Mar;20(2):351-60
Sporadic corticobasal syndrome due to FTLD-TDP.
Tartaglia MC, Sidhu M, Laluz V, Racine C, Rabinovici GD, Creighton K, Karydas A, Rademakers R, Huang EJ, Miller BL, DeArmond SJ, Seeley WW
Acta neuropathologica 2010 Mar;119(3):365-74
Acta neuropathologica 2010 Mar;119(3):365-74
Transgenic rat model of neurodegeneration caused by mutation in the TDP gene.
Zhou H, Huang C, Chen H, Wang D, Landel CP, Xia PY, Bowser R, Liu YJ, Xia XG
PLoS genetics 2010 Mar 26;6(3):e1000887
PLoS genetics 2010 Mar 26;6(3):e1000887
Loss of ALS2/Alsin exacerbates motor dysfunction in a SOD1-expressing mouse ALS model by disturbing endolysosomal trafficking.
Hadano S, Otomo A, Kunita R, Suzuki-Utsunomiya K, Akatsuka A, Koike M, Aoki M, Uchiyama Y, Itoyama Y, Ikeda JE
PloS one 2010 Mar 22;5(3):e9805
PloS one 2010 Mar 22;5(3):e9805
FUS mutations in amyotrophic lateral sclerosis: clinical, pathological, neurophysiological and genetic analysis.
Blair IP, Williams KL, Warraich ST, Durnall JC, Thoeng AD, Manavis J, Blumbergs PC, Vucic S, Kiernan MC, Nicholson GA
Journal of neurology, neurosurgery, and psychiatry 2010 Jun;81(6):639-45
Journal of neurology, neurosurgery, and psychiatry 2010 Jun;81(6):639-45
FUS-immunoreactive inclusions are a common feature in sporadic and non-SOD1 familial amyotrophic lateral sclerosis.
Deng HX, Zhai H, Bigio EH, Yan J, Fecto F, Ajroud K, Mishra M, Ajroud-Driss S, Heller S, Sufit R, Siddique N, Mugnaini E, Siddique T
Annals of neurology 2010 Jun;67(6):739-48
Annals of neurology 2010 Jun;67(6):739-48
Relationship between Bunina bodies and TDP-43 inclusions in spinal anterior horn in amyotrophic lateral sclerosis.
Mori F, Tanji K, Miki Y, Kakita A, Takahashi H, Wakabayashi K
Neuropathology and applied neurobiology 2010 Jun;36(4):345-52
Neuropathology and applied neurobiology 2010 Jun;36(4):345-52
International Workshop on Inclusion Body Myositis held at the Institute of Myology, Paris, on 29 May 2009.
Benveniste O, Hilton-Jones D
Neuromuscular disorders : NMD 2010 Jun;20(6):414-21
Neuromuscular disorders : NMD 2010 Jun;20(6):414-21
Nuclear import impairment causes cytoplasmic trans-activation response DNA-binding protein accumulation and is associated with frontotemporal lobar degeneration.
Nishimura AL, Zupunski V, Troakes C, Kathe C, Fratta P, Howell M, Gallo JM, Hortobágyi T, Shaw CE, Rogelj B
Brain : a journal of neurology 2010 Jun;133(Pt 6):1763-71
Brain : a journal of neurology 2010 Jun;133(Pt 6):1763-71
TDP-43 mediates degeneration in a novel Drosophila model of disease caused by mutations in VCP/p97.
Ritson GP, Custer SK, Freibaum BD, Guinto JB, Geffel D, Moore J, Tang W, Winton MJ, Neumann M, Trojanowski JQ, Lee VM, Forman MS, Taylor JP
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Jun 2;30(22):7729-39
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Jun 2;30(22):7729-39
Neuropathology of olfactory ensheathing cell transplantation into the brain of two amyotrophic lateral sclerosis (ALS) patients.
Giordana MT, Grifoni S, Votta B, Magistrello M, Vercellino M, Pellerino A, Navone R, Valentini C, Calvo A, Chiò A
Brain pathology (Zurich, Switzerland) 2010 Jul;20(4):730-7
Brain pathology (Zurich, Switzerland) 2010 Jul;20(4):730-7
Accelerated lipofuscinosis and ubiquitination in granulin knockout mice suggest a role for progranulin in successful aging.
Ahmed Z, Sheng H, Xu YF, Lin WL, Innes AE, Gass J, Yu X, Wuertzer CA, Hou H, Chiba S, Yamanouchi K, Leissring M, Petrucelli L, Nishihara M, Hutton ML, McGowan E, Dickson DW, Lewis J
The American journal of pathology 2010 Jul;177(1):311-24
The American journal of pathology 2010 Jul;177(1):311-24
Caudate atrophy on MRI is a characteristic feature of FTLD-FUS.
Josephs KA, Whitwell JL, Parisi JE, Petersen RC, Boeve BF, Jack CR Jr, Dickson DW
European journal of neurology 2010 Jul;17(7):969-75
European journal of neurology 2010 Jul;17(7):969-75
TDP-43 pathology in sporadic ALS occurs in motor neurons lacking the RNA editing enzyme ADAR2.
Aizawa H, Sawada J, Hideyama T, Yamashita T, Katayama T, Hasebe N, Kimura T, Yahara O, Kwak S
Acta neuropathologica 2010 Jul;120(1):75-84
Acta neuropathologica 2010 Jul;120(1):75-84
TDP-43 pathology in primary progressive aphasia and frontotemporal dementia with pathologic Alzheimer disease.
Bigio EH, Mishra M, Hatanpaa KJ, White CL 3rd, Johnson N, Rademaker A, Weitner BB, Deng HX, Dubner SD, Weintraub S, Mesulam M
Acta neuropathologica 2010 Jul;120(1):43-54
Acta neuropathologica 2010 Jul;120(1):43-54
Alterations in subcellular localization of TDP-43 immunoreactivity in the anterior horns in sporadic amyotrophic lateral sclerosis.
Sasaki S, Takeda T, Shibata N, Kobayashi M
Neuroscience letters 2010 Jul 5;478(2):72-6
Neuroscience letters 2010 Jul 5;478(2):72-6
Sustained expression of TDP-43 and FUS in motor neurons in rodent's lifetime.
Huang C, Xia PY, Zhou H
International journal of biological sciences 2010 Jul 4;6(4):396-406
International journal of biological sciences 2010 Jul 4;6(4):396-406
A novel X-linked four-repeat tauopathy with Parkinsonism and spasticity.
Poorkaj P, Raskind WH, Leverenz JB, Matsushita M, Zabetian CP, Samii A, Kim S, Gazi N, Nutt JG, Wolff J, Yearout D, Greenup JL, Steinbart EJ, Bird TD
Movement disorders : official journal of the Movement Disorder Society 2010 Jul 30;25(10):1409-17
Movement disorders : official journal of the Movement Disorder Society 2010 Jul 30;25(10):1409-17
ALS-associated mutations in TDP-43 increase its stability and promote TDP-43 complexes with FUS/TLS.
Ling SC, Albuquerque CP, Han JS, Lagier-Tourenne C, Tokunaga S, Zhou H, Cleveland DW
Proceedings of the National Academy of Sciences of the United States of America 2010 Jul 27;107(30):13318-23
Proceedings of the National Academy of Sciences of the United States of America 2010 Jul 27;107(30):13318-23
Anatomical differences between CBS-corticobasal degeneration and CBS-Alzheimer's disease.
Josephs KA, Whitwell JL, Boeve BF, Knopman DS, Petersen RC, Hu WT, Parisi JE, Dickson DW, Jack CR Jr
Movement disorders : official journal of the Movement Disorder Society 2010 Jul 15;25(9):1246-52
Movement disorders : official journal of the Movement Disorder Society 2010 Jul 15;25(9):1246-52
Modeling the association between 43 different clinical and pathological variables and the severity of cognitive impairment in a large autopsy cohort of elderly persons.
Nelson PT, Abner EL, Schmitt FA, Kryscio RJ, Jicha GA, Smith CD, Davis DG, Poduska JW, Patel E, Mendiondo MS, Markesbery WR
Brain pathology (Zurich, Switzerland) 2010 Jan;20(1):66-79
Brain pathology (Zurich, Switzerland) 2010 Jan;20(1):66-79
Semantic dementia: demography, familial factors and survival in a consecutive series of 100 cases.
Hodges JR, Mitchell J, Dawson K, Spillantini MG, Xuereb JH, McMonagle P, Nestor PJ, Patterson K
Brain : a journal of neurology 2010 Jan;133(Pt 1):300-6
Brain : a journal of neurology 2010 Jan;133(Pt 1):300-6
Brain progranulin expression in GRN-associated frontotemporal lobar degeneration.
Chen-Plotkin AS, Xiao J, Geser F, Martinez-Lage M, Grossman M, Unger T, Wood EM, Van Deerlin VM, Trojanowski JQ, Lee VM
Acta neuropathologica 2010 Jan;119(1):111-22
Acta neuropathologica 2010 Jan;119(1):111-22
Knockdown of transactive response DNA-binding protein (TDP-43) downregulates histone deacetylase 6.
Fiesel FC, Voigt A, Weber SS, Van den Haute C, Waldenmaier A, Görner K, Walter M, Anderson ML, Kern JV, Rasse TM, Schmidt T, Springer W, Kirchner R, Bonin M, Neumann M, Baekelandt V, Alunni-Fabbroni M, Schulz JB, Kahle PJ
The EMBO journal 2010 Jan 6;29(1):209-21
The EMBO journal 2010 Jan 6;29(1):209-21
In vivo amyloid imaging in autopsy-confirmed Parkinson disease with dementia.
Burack MA, Hartlein J, Flores HP, Taylor-Reinwald L, Perlmutter JS, Cairns NJ
Neurology 2010 Jan 5;74(1):77-84
Neurology 2010 Jan 5;74(1):77-84
Exaggerated inflammation, impaired host defense, and neuropathology in progranulin-deficient mice.
Yin F, Banerjee R, Thomas B, Zhou P, Qian L, Jia T, Ma X, Ma Y, Iadecola C, Beal MF, Nathan C, Ding A
The Journal of experimental medicine 2010 Jan 18;207(1):117-28
The Journal of experimental medicine 2010 Jan 18;207(1):117-28
Cytoplasmic mislocalization of TDP-43 is toxic to neurons and enhanced by a mutation associated with familial amyotrophic lateral sclerosis.
Barmada SJ, Skibinski G, Korb E, Rao EJ, Wu JY, Finkbeiner S
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Jan 13;30(2):639-49
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Jan 13;30(2):639-49
Characterization of alternative isoforms and inclusion body of the TAR DNA-binding protein-43.
Nishimoto Y, Ito D, Yagi T, Nihei Y, Tsunoda Y, Suzuki N
The Journal of biological chemistry 2010 Jan 1;285(1):608-19
The Journal of biological chemistry 2010 Jan 1;285(1):608-19
Alzheimer disease-like phenotype associated with the c.154delA mutation in progranulin.
Kelley BJ, Haidar W, Boeve BF, Baker M, Shiung M, Knopman DS, Rademakers R, Hutton M, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Petersen RC
Archives of neurology 2010 Feb;67(2):171-7
Archives of neurology 2010 Feb;67(2):171-7
Neuropathological heterogeneity in frontotemporal lobar degeneration with TDP-43 proteinopathy: a quantitative study of 94 cases using principal components analysis.
Armstrong RA, Ellis W, Hamilton RL, Mackenzie IR, Hedreen J, Gearing M, Montine T, Vonsattel JP, Head E, Lieberman AP, Cairns NJ
Journal of neural transmission (Vienna, Austria : 1996) 2010 Feb;117(2):227-39
Journal of neural transmission (Vienna, Austria : 1996) 2010 Feb;117(2):227-39
Global analysis of TDP-43 interacting proteins reveals strong association with RNA splicing and translation machinery.
Freibaum BD, Chitta RK, High AA, Taylor JP
Journal of proteome research 2010 Feb 5;9(2):1104-20
Journal of proteome research 2010 Feb 5;9(2):1104-20
TDP-43 is a developmentally regulated protein essential for early embryonic development.
Sephton CF, Good SK, Atkin S, Dewey CM, Mayer P 3rd, Herz J, Yu G
The Journal of biological chemistry 2010 Feb 26;285(9):6826-34
The Journal of biological chemistry 2010 Feb 26;285(9):6826-34
TDP-43 transgenic mice develop spastic paralysis and neuronal inclusions characteristic of ALS and frontotemporal lobar degeneration.
Wils H, Kleinberger G, Janssens J, Pereson S, Joris G, Cuijt I, Smits V, Ceuterick-de Groote C, Van Broeckhoven C, Kumar-Singh S
Proceedings of the National Academy of Sciences of the United States of America 2010 Feb 23;107(8):3858-63
Proceedings of the National Academy of Sciences of the United States of America 2010 Feb 23;107(8):3858-63
Gain and loss of function of ALS-related mutations of TARDBP (TDP-43) cause motor deficits in vivo.
Kabashi E, Lin L, Tradewell ML, Dion PA, Bercier V, Bourgouin P, Rochefort D, Bel Hadj S, Durham HD, Vande Velde C, Rouleau GA, Drapeau P
Human molecular genetics 2010 Feb 15;19(4):671-83
Human molecular genetics 2010 Feb 15;19(4):671-83
Pathologic evidence that the T188R mutation in PRNP is associated with prion disease.
Tartaglia MC, Thai JN, See T, Kuo A, Harbaugh R, Raudabaugh B, Cali I, Sattavat M, Sanchez H, DeArmond SJ, Geschwind MD
Journal of neuropathology and experimental neurology 2010 Dec;69(12):1220-7
Journal of neuropathology and experimental neurology 2010 Dec;69(12):1220-7
TDP-43 physically interacts with amyotrophic lateral sclerosis-linked mutant CuZn superoxide dismutase.
Higashi S, Tsuchiya Y, Araki T, Wada K, Kabuta T
Neurochemistry international 2010 Dec;57(8):906-13
Neurochemistry international 2010 Dec;57(8):906-13
TDP-43 proteinopathy in familial motor neurone disease with TARDBP A315T mutation: a case report.
Cairns NJ, Perrin RJ, Schmidt RE, Gru A, Green KG, Carter D, Taylor-Reinwald L, Morris JC, Gitcho MA, Baloh RH
Neuropathology and applied neurobiology 2010 Dec;36(7):673-9
Neuropathology and applied neurobiology 2010 Dec;36(7):673-9
Widespread occurrence of eosinophilic neuronal cytoplasmic inclusions in an asymptomatic adult: a novel ubiquitin-negative filamentous inclusion.
Mori F, Miki Y, Tanji K, Kusumi T, Kijima H, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Dec;30(6):648-53
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Dec;30(6):648-53
Very early-onset frontotemporal dementia with no family history predicts underlying fused in sarcoma pathology.
Loy CT, McCusker E, Kril JJ, Kwok JB, Brooks WS, McCann H, Isaacs AM, Halliday GM
Brain : a journal of neurology 2010 Dec;133(Pt 12):e158; author reply e159
Brain : a journal of neurology 2010 Dec;133(Pt 12):e158; author reply e159
Exome sequencing reveals VCP mutations as a cause of familial ALS.
Johnson JO, Mandrioli J, Benatar M, Abramzon Y, Van Deerlin VM, Trojanowski JQ, Gibbs JR, Brunetti M, Gronka S, Wuu J, Ding J, McCluskey L, Martinez-Lage M, Falcone D, Hernandez DG, Arepalli S, Chong S, Schymick JC, Rothstein J, Landi F, Wang YD, Calvo A, Mora G, Sabatelli M, Monsurrò MR, Battistini S, Salvi F, Spataro R, Sola P, Borghero G, ITALSGEN Consortium, Galassi G, Scholz SW, Taylor JP, Restagno G, Chiò A, Traynor BJ
Neuron 2010 Dec 9;68(5):857-64
Neuron 2010 Dec 9;68(5):857-64
The C-terminal TDP-43 fragments have a high aggregation propensity and harm neurons by a dominant-negative mechanism.
Yang C, Tan W, Whittle C, Qiu L, Cao L, Akbarian S, Xu Z
PloS one 2010 Dec 31;5(12):e15878
PloS one 2010 Dec 31;5(12):e15878
Phosphoproteomic analysis reveals site-specific changes in GFAP and NDRG2 phosphorylation in frontotemporal lobar degeneration.
Herskowitz JH, Seyfried NT, Duong DM, Xia Q, Rees HD, Gearing M, Peng J, Lah JJ, Levey AI
Journal of proteome research 2010 Dec 3;9(12):6368-79
Journal of proteome research 2010 Dec 3;9(12):6368-79
Progranulin deficiency leads to enhanced cell vulnerability and TDP-43 translocation in primary neuronal cultures.
Guo A, Tapia L, Bamji SX, Cynader MS, Jia W
Brain research 2010 Dec 17;1366:1-8
Brain research 2010 Dec 17;1366:1-8
Does TDP-43 type confer a distinct pattern of atrophy in frontotemporal lobar degeneration?
Whitwell JL, Jack CR Jr, Parisi JE, Senjem ML, Knopman DS, Boeve BF, Rademakers R, Baker M, Petersen RC, Dickson DW, Josephs KA
Neurology 2010 Dec 14;75(24):2212-20
Neurology 2010 Dec 14;75(24):2212-20
TDP-43 subtypes are associated with distinct atrophy patterns in frontotemporal dementia.
Rohrer JD, Geser F, Zhou J, Gennatas ED, Sidhu M, Trojanowski JQ, Dearmond SJ, Miller BL, Seeley WW
Neurology 2010 Dec 14;75(24):2204-11
Neurology 2010 Dec 14;75(24):2204-11
Fus gene mutations in familial and sporadic amyotrophic lateral sclerosis.
Rademakers R, Stewart H, Dejesus-Hernandez M, Krieger C, Graff-Radford N, Fabros M, Briemberg H, Cashman N, Eisen A, Mackenzie IR
Muscle & nerve 2010 Aug;42(2):170-6
Muscle & nerve 2010 Aug;42(2):170-6
Abnormal TDP-43 expression is identified in the neocortex in cases of dementia pugilistica, but is mainly confined to the limbic system when identified in high and moderate stages of Alzheimer's disease.
King A, Sweeney F, Bodi I, Troakes C, Maekawa S, Al-Sarraj S
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Aug;30(4):408-19
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Aug;30(4):408-19
Pseudopolyneuritic form of ALS revisited: clinical and pathological heterogeneity.
Kobayashi Z, Tsuchiya K, Arai T, Yokota O, Watabiki S, Ishizu H, Akiyama H, Mizusawa H
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Aug;30(4):372-80
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Aug;30(4):372-80
Ataxin-2 intermediate-length polyglutamine expansions are associated with increased risk for ALS.
Elden AC, Kim HJ, Hart MP, Chen-Plotkin AS, Johnson BS, Fang X, Armakola M, Geser F, Greene R, Lu MM, Padmanabhan A, Clay-Falcone D, McCluskey L, Elman L, Juhr D, Gruber PJ, Rüb U, Auburger G, Trojanowski JQ, Lee VM, Van Deerlin VM, Bonini NM, Gitler AD
Nature 2010 Aug 26;466(7310):1069-75
Nature 2010 Aug 26;466(7310):1069-75
Interaction with polyglutamine aggregates reveals a Q/N-rich domain in TDP-43.
Fuentealba RA, Udan M, Bell S, Wegorzewska I, Shao J, Diamond MI, Weihl CC, Baloh RH
The Journal of biological chemistry 2010 Aug 20;285(34):26304-14
The Journal of biological chemistry 2010 Aug 20;285(34):26304-14
Elevated expression of TDP-43 in the forebrain of mice is sufficient to cause neurological and pathological phenotypes mimicking FTLD-U.
Tsai KJ, Yang CH, Fang YH, Cho KH, Chien WL, Wang WT, Wu TW, Lin CP, Fu WM, Shen CK
The Journal of experimental medicine 2010 Aug 2;207(8):1661-73
The Journal of experimental medicine 2010 Aug 2;207(8):1661-73
TDP-43-mediated neuron loss in vivo requires RNA-binding activity.
Voigt A, Herholz D, Fiesel FC, Kaur K, Müller D, Karsten P, Weber SS, Kahle PJ, Marquardt T, Schulz JB
PloS one 2010 Aug 18;5(8):e12247
PloS one 2010 Aug 18;5(8):e12247
Juvenile ALS with basophilic inclusions is a FUS proteinopathy with FUS mutations.
Bäumer D, Hilton D, Paine SM, Turner MR, Lowe J, Talbot K, Ansorge O
Neurology 2010 Aug 17;75(7):611-8
Neurology 2010 Aug 17;75(7):611-8
Neurotoxic effects of TDP-43 overexpression in C. elegans.
Ash PE, Zhang YJ, Roberts CM, Saldi T, Hutter H, Buratti E, Petrucelli L, Link CD
Human molecular genetics 2010 Aug 15;19(16):3206-18
Human molecular genetics 2010 Aug 15;19(16):3206-18
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
Xu YF, Gendron TF, Zhang YJ, Lin WL, D'Alton S, Sheng H, Casey MC, Tong J, Knight J, Yu X, Rademakers R, Boylan K, Hutton M, McGowan E, Dickson DW, Lewis J, Petrucelli L
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Aug 11;30(32):10851-9
The Journal of neuroscience : the official journal of the Society for Neuroscience 2010 Aug 11;30(32):10851-9
Multiplex SILAC analysis of a cellular TDP-43 proteinopathy model reveals protein inclusions associated with SUMOylation and diverse polyubiquitin chains.
Seyfried NT, Gozal YM, Dammer EB, Xia Q, Duong DM, Cheng D, Lah JJ, Levey AI, Peng J
Molecular & cellular proteomics : MCP 2010 Apr;9(4):705-18
Molecular & cellular proteomics : MCP 2010 Apr;9(4):705-18
Survival in progressive supranuclear palsy and frontotemporal dementia.
Chiu WZ, Kaat LD, Seelaar H, Rosso SM, Boon AJ, Kamphorst W, van Swieten JC
Journal of neurology, neurosurgery, and psychiatry 2010 Apr;81(4):441-5
Journal of neurology, neurosurgery, and psychiatry 2010 Apr;81(4):441-5
Olfactory epithelium amyloid-beta and paired helical filament-tau pathology in Alzheimer disease.
Arnold SE, Lee EB, Moberg PJ, Stutzbach L, Kazi H, Han LY, Lee VM, Trojanowski JQ
Annals of neurology 2010 Apr;67(4):462-9
Annals of neurology 2010 Apr;67(4):462-9
FALS with FUS mutation in Japan, with early onset, rapid progress and basophilic inclusion.
Suzuki N, Aoki M, Warita H, Kato M, Mizuno H, Shimakura N, Akiyama T, Furuya H, Hokonohara T, Iwaki A, Togashi S, Konno H, Itoyama Y
Journal of human genetics 2010 Apr;55(4):252-4
Journal of human genetics 2010 Apr;55(4):252-4
Sporadic amyotrophic lateral sclerosis with pallido-nigro-luysian degeneration: a TDP-43 immunohistochemical study.
Miki Y, Mori F, Nunomura J, Ookawa K, Yajima N, Yagihashi S, Wakabayashi K
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Apr;30(2):149-53
Neuropathology : official journal of the Japanese Society of Neuropathology 2010 Apr;30(2):149-53
Alteration of biochemical and pathological properties of TDP-43 protein by a lipid mediator, 15-deoxy-Delta(12,14)-prostaglandin J(2).
Zhang HX, Tanji K, Yoshida H, Hayakari M, Shibata T, Mori F, Uchida K, Wakabayashi K
Experimental neurology 2010 Apr;222(2):296-303
Experimental neurology 2010 Apr;222(2):296-303
Alzheimer's disease phenotypes and genotypes associated with mutations in presenilin 2.
Jayadev S, Leverenz JB, Steinbart E, Stahl J, Klunk W, Yu CE, Bird TD
Brain : a journal of neurology 2010 Apr;133(Pt 4):1143-54
Brain : a journal of neurology 2010 Apr;133(Pt 4):1143-54
Loss of murine TDP-43 disrupts motor function and plays an essential role in embryogenesis.
Kraemer BC, Schuck T, Wheeler JM, Robinson LC, Trojanowski JQ, Lee VM, Schellenberg GD
Acta neuropathologica 2010 Apr;119(4):409-19
Acta neuropathologica 2010 Apr;119(4):409-19
Ubiquilin modifies TDP-43 toxicity in a Drosophila model of amyotrophic lateral sclerosis (ALS).
Hanson KA, Kim SH, Wassarman DA, Tibbetts RS
The Journal of biological chemistry 2010 Apr 9;285(15):11068-72
The Journal of biological chemistry 2010 Apr 9;285(15):11068-72
Familial amyotrophic lateral sclerosis is associated with a mutation in D-amino acid oxidase.
Mitchell J, Paul P, Chen HJ, Morris A, Payling M, Falchi M, Habgood J, Panoutsou S, Winkler S, Tisato V, Hajitou A, Smith B, Vance C, Shaw C, Mazarakis ND, de Belleroche J
Proceedings of the National Academy of Sciences of the United States of America 2010 Apr 20;107(16):7556-61
Proceedings of the National Academy of Sciences of the United States of America 2010 Apr 20;107(16):7556-61
Neuropathological investigation of regions responsible for semantic aphasia in frontotemporal lobar degeneration.
Yamamoto R, Iseki E, Higashi S, Murayama N, Minegishi M, Sato K, Hino H, Fujisawa K, Kosaka K, Togo T, Katsuse O, Uchikado H, Furukawa Y, Yoshida M, Hashizume Y, Arai H
Dementia and geriatric cognitive disorders 2009;27(3):214-23
Dementia and geriatric cognitive disorders 2009;27(3):214-23
Abnormal localization of leucine-rich repeat kinase 2 to the endosomal-lysosomal compartment in lewy body disease.
Higashi S, Moore DJ, Yamamoto R, Minegishi M, Sato K, Togo T, Katsuse O, Uchikado H, Furukawa Y, Hino H, Kosaka K, Emson PC, Wada K, Dawson VL, Dawson TM, Arai H, Iseki E
Journal of neuropathology and experimental neurology 2009 Sep;68(9):994-1005
Journal of neuropathology and experimental neurology 2009 Sep;68(9):994-1005
TDP-43 localizes in mRNA transcription and processing sites in mammalian neurons.
Casafont I, Bengoechea R, Tapia O, Berciano MT, Lafarga M
Journal of structural biology 2009 Sep;167(3):235-41
Journal of structural biology 2009 Sep;167(3):235-41
TDP-43 in ubiquitinated inclusions in the inferior olives in frontotemporal lobar degeneration and in other neurodegenerative diseases: a degenerative process distinct from normal ageing.
Davidson Y, Amin H, Kelley T, Shi J, Tian J, Kumaran R, Lashley T, Lees AJ, DuPlessis D, Neary D, Snowden J, Akiyama H, Arai T, Hasegawa M, Bandopadhyay R, Sikkink S, Pickering-Brown S, Mann DM
Acta neuropathologica 2009 Sep;118(3):359-69
Acta neuropathologica 2009 Sep;118(3):359-69
Evaluation of subcortical pathology and clinical correlations in FTLD-U subtypes.
Josephs KA, Stroh A, Dugger B, Dickson DW
Acta neuropathologica 2009 Sep;118(3):349-58
Acta neuropathologica 2009 Sep;118(3):349-58
Chemical manipulation of hsp70 ATPase activity regulates tau stability.
Jinwal UK, Miyata Y, Koren J 3rd, Jones JR, Trotter JH, Chang L, O'Leary J, Morgan D, Lee DC, Shults CL, Rousaki A, Weeber EJ, Zuiderweg ER, Gestwicki JE, Dickey CA
The Journal of neuroscience : the official journal of the Society for Neuroscience 2009 Sep 30;29(39):12079-88
The Journal of neuroscience : the official journal of the Society for Neuroscience 2009 Sep 30;29(39):12079-88
Phosphorylation-dependent TDP-43 antibody detects intraneuronal dot-like structures showing morphological characters of granulovacuolar degeneration.
Kadokura A, Yamazaki T, Kakuda S, Makioka K, Lemere CA, Fujita Y, Takatama M, Okamoto K
Neuroscience letters 2009 Sep 29;463(1):87-92
Neuroscience letters 2009 Sep 29;463(1):87-92
Frontotemporal dementia and amyotrophic lateral sclerosis-associated disease protein TDP-43 promotes dendritic branching.
Lu Y, Ferris J, Gao FB
Molecular brain 2009 Sep 25;2:30
Molecular brain 2009 Sep 25;2:30
TARDBP variation associated with frontotemporal dementia, supranuclear gaze palsy, and chorea.
Kovacs GG, Murrell JR, Horvath S, Haraszti L, Majtenyi K, Molnar MJ, Budka H, Ghetti B, Spina S
Movement disorders : official journal of the Movement Disorder Society 2009 Sep 15;24(12):1843-7
Movement disorders : official journal of the Movement Disorder Society 2009 Sep 15;24(12):1843-7
Truncation and pathogenic mutations facilitate the formation of intracellular aggregates of TDP-43.
Nonaka T, Kametani F, Arai T, Akiyama H, Hasegawa M
Human molecular genetics 2009 Sep 15;18(18):3353-64
Human molecular genetics 2009 Sep 15;18(18):3353-64
Regional distribution of TDP-43 inclusions in Alzheimer disease (AD) brains: their relation to AD common pathology.
Kadokura A, Yamazaki T, Lemere CA, Takatama M, Okamoto K
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Oct;29(5):566-73
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Oct;29(5):566-73
Accumulation of TDP-43 and alpha-actin in an amyotrophic lateral sclerosis patient with the K17I ANG mutation.
Seilhean D, Cazeneuve C, Thuriès V, Russaouen O, Millecamps S, Salachas F, Meininger V, Leguern E, Duyckaerts C
Acta neuropathologica 2009 Oct;118(4):561-73
Acta neuropathologica 2009 Oct;118(4):561-73
Selective occurrence of TDP-43-immunoreactive inclusions in the lower motor neurons in Machado-Joseph disease.
Tan CF, Yamada M, Toyoshima Y, Yokoseki A, Miki Y, Hoshi Y, Kaneko H, Ikeuchi T, Onodera O, Kakita A, Takahashi H
Acta neuropathologica 2009 Oct;118(4):553-60
Acta neuropathologica 2009 Oct;118(4):553-60
Progranulin is expressed within motor neurons and promotes neuronal cell survival.
Ryan CL, Baranowski DC, Chitramuthu BP, Malik S, Li Z, Cao M, Minotti S, Durham HD, Kay DG, Shaw CA, Bennett HP, Bateman A
BMC neuroscience 2009 Oct 27;10:130
BMC neuroscience 2009 Oct 27;10:130
Decreased cystatin C immunoreactivity in spinal motor neurons and astrocytes in amyotrophic lateral sclerosis.
Mori F, Tanji K, Miki Y, Wakabayashi K
Journal of neuropathology and experimental neurology 2009 Nov;68(11):1200-6
Journal of neuropathology and experimental neurology 2009 Nov;68(11):1200-6
Transactivation response DNA-binding protein 43 microvasculopathy in frontotemporal degeneration and familial Lewy body disease.
Lin WL, Castanedes-Casey M, Dickson DW
Journal of neuropathology and experimental neurology 2009 Nov;68(11):1167-76
Journal of neuropathology and experimental neurology 2009 Nov;68(11):1167-76
A new subtype of frontotemporal lobar degeneration with FUS pathology.
Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar HA, Mackenzie IR
Brain : a journal of neurology 2009 Nov;132(Pt 11):2922-31
Brain : a journal of neurology 2009 Nov;132(Pt 11):2922-31
Plasma phosphorylated-TDP-43 protein levels correlate with brain pathology in frontotemporal lobar degeneration.
Foulds PG, Davidson Y, Mishra M, Hobson DJ, Humphreys KM, Taylor M, Johnson N, Weintraub S, Akiyama H, Arai T, Hasegawa M, Bigio EH, Benson FE, Allsop D, Mann DM
Acta neuropathologica 2009 Nov;118(5):647-58
Acta neuropathologica 2009 Nov;118(5):647-58
TARDBP 3'-UTR variant in autopsy-confirmed frontotemporal lobar degeneration with TDP-43 proteinopathy.
Gitcho MA, Bigio EH, Mishra M, Johnson N, Weintraub S, Mesulam M, Rademakers R, Chakraverty S, Cruchaga C, Morris JC, Goate AM, Cairns NJ
Acta neuropathologica 2009 Nov;118(5):633-45
Acta neuropathologica 2009 Nov;118(5):633-45
Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease.
Neumann M, Roeber S, Kretzschmar HA, Rademakers R, Baker M, Mackenzie IR
Acta neuropathologica 2009 Nov;118(5):605-16
Acta neuropathologica 2009 Nov;118(5):605-16
TDP-43 is recruited to stress granules in conditions of oxidative insult.
Colombrita C, Zennaro E, Fallini C, Weber M, Sommacal A, Buratti E, Silani V, Ratti A
Journal of neurochemistry 2009 Nov;111(4):1051-61
Journal of neurochemistry 2009 Nov;111(4):1051-61
Cytosolic TDP-43 expression following axotomy is associated with caspase 3 activation in NFL-/- mice: support for a role for TDP-43 in the physiological response to neuronal injury.
Moisse K, Mepham J, Volkening K, Welch I, Hill T, Strong MJ
Brain research 2009 Nov 3;1296:176-86
Brain research 2009 Nov 3;1296:176-86
TDP-43 mutant transgenic mice develop features of ALS and frontotemporal lobar degeneration.
Wegorzewska I, Bell S, Cairns NJ, Miller TM, Baloh RH
Proceedings of the National Academy of Sciences of the United States of America 2009 Nov 3;106(44):18809-14
Proceedings of the National Academy of Sciences of the United States of America 2009 Nov 3;106(44):18809-14
Prominent phenotypic variability associated with mutations in Progranulin.
Kelley BJ, Haidar W, Boeve BF, Baker M, Graff-Radford NR, Krefft T, Frank AR, Jack CR Jr, Shiung M, Knopman DS, Josephs KA, Parashos SA, Rademakers R, Hutton M, Pickering-Brown S, Adamson J, Kuntz KM, Dickson DW, Parisi JE, Smith GE, Ivnik RJ, Petersen RC
Neurobiology of aging 2009 May;30(5):739-51
Neurobiology of aging 2009 May;30(5):739-51
Pallidonigral TDP-43 pathology in Perry syndrome.
Wider C, Dickson DW, Stoessl AJ, Tsuboi Y, Chapon F, Gutmann L, Lechevalier B, Calne DB, Personett DA, Hulihan M, Kachergus J, Rademakers R, Baker MC, Grantier LL, Sujith OK, Brown L, Calne S, Farrer MJ, Wszolek ZK
Parkinsonism & related disorders 2009 May;15(4):281-6
Parkinsonism & related disorders 2009 May;15(4):281-6
VCP mutations causing frontotemporal lobar degeneration disrupt localization of TDP-43 and induce cell death.
Gitcho MA, Strider J, Carter D, Taylor-Reinwald L, Forman MS, Goate AM, Cairns NJ
The Journal of biological chemistry 2009 May 1;284(18):12384-98
The Journal of biological chemistry 2009 May 1;284(18):12384-98
TDP-43 is not present in brain tissue of patients with schizophrenia.
Mateen FJ, Josephs KA
Schizophrenia research 2009 Mar;108(1-3):297-8
Schizophrenia research 2009 Mar;108(1-3):297-8
Expression of TDP-43 C-terminal Fragments in Vitro Recapitulates Pathological Features of TDP-43 Proteinopathies.
Igaz LM, Kwong LK, Chen-Plotkin A, Winton MJ, Unger TL, Xu Y, Neumann M, Trojanowski JQ, Lee VM
The Journal of biological chemistry 2009 Mar 27;284(13):8516-24
The Journal of biological chemistry 2009 Mar 27;284(13):8516-24
Potentiation of amyotrophic lateral sclerosis (ALS)-associated TDP-43 aggregation by the proteasome-targeting factor, ubiquilin 1.
Kim SH, Shi Y, Hanson KA, Williams LM, Sakasai R, Bowler MJ, Tibbetts RS
The Journal of biological chemistry 2009 Mar 20;284(12):8083-92
The Journal of biological chemistry 2009 Mar 20;284(12):8083-92
Inflammatory myopathies with mitochondrial pathology and protein aggregates.
Temiz P, Weihl CC, Pestronk A
Journal of the neurological sciences 2009 Mar 15;278(1-2):25-9
Journal of the neurological sciences 2009 Mar 15;278(1-2):25-9
Hippocampal sclerosis with four-repeat tau-positive round inclusions in the dentate gyrus: a new type of four-repeat tauopathy.
Miki Y, Mori F, Hori E, Kaimori M, Wakabayashi K
Acta neuropathologica 2009 Jun;117(6):713-8
Acta neuropathologica 2009 Jun;117(6):713-8
Sarcoplasmic redistribution of nuclear TDP-43 in inclusion body myositis.
Salajegheh M, Pinkus JL, Taylor JP, Amato AA, Nazareno R, Baloh RH, Greenberg SA
Muscle & nerve 2009 Jul;40(1):19-31
Muscle & nerve 2009 Jul;40(1):19-31
Deep brain stimulation in dystonia: sonographic monitoring of electrode placement into the globus pallidus internus.
Walter U, Wolters A, Wittstock M, Benecke R, Schroeder HW, Müller JU
Movement disorders : official journal of the Movement Disorder Society 2009 Jul 30;24(10):1538-41
Movement disorders : official journal of the Movement Disorder Society 2009 Jul 30;24(10):1538-41
No Lewy pathology in monkeys with over 10 years of severe MPTP Parkinsonism.
Halliday G, Herrero MT, Murphy K, McCann H, Ros-Bernal F, Barcia C, Mori H, Blesa FJ, Obeso JA
Movement disorders : official journal of the Movement Disorder Society 2009 Jul 30;24(10):1519-23
Movement disorders : official journal of the Movement Disorder Society 2009 Jul 30;24(10):1519-23
Methylene blue and dimebon inhibit aggregation of TDP-43 in cellular models.
Yamashita M, Nonaka T, Arai T, Kametani F, Buchman VL, Ninkina N, Bachurin SO, Akiyama H, Goedert M, Hasegawa M
FEBS letters 2009 Jul 21;583(14):2419-24
FEBS letters 2009 Jul 21;583(14):2419-24
Mislocalization of TDP-43 in the G93A mutant SOD1 transgenic mouse model of ALS.
Shan X, Vocadlo D, Krieger C
Neuroscience letters 2009 Jul 17;458(2):70-4
Neuroscience letters 2009 Jul 17;458(2):70-4
Nuclear TAR DNA binding protein 43 expression in spinal cord neurons correlates with the clinical course in amyotrophic lateral sclerosis.
Sumi H, Kato S, Mochimaru Y, Fujimura H, Etoh M, Sakoda S
Journal of neuropathology and experimental neurology 2009 Jan;68(1):37-47
Journal of neuropathology and experimental neurology 2009 Jan;68(1):37-47
Abstracts of the 110th Meeting of the British Neuropathological Society. January 7-9, 2009. London, United Kingdom.
Neuropathology and applied neurobiology 2009 Jan;35 Suppl 1:1-38
Increased TDP-43 protein in cerebrospinal fluid of patients with amyotrophic lateral sclerosis.
Kasai T, Tokuda T, Ishigami N, Sasayama H, Foulds P, Mitchell DJ, Mann DM, Allsop D, Nakagawa M
Acta neuropathologica 2009 Jan;117(1):55-62
Acta neuropathologica 2009 Jan;117(1):55-62
Sporadic amyotrophic lateral sclerosis of long duration is associated with relatively mild TDP-43 pathology.
Nishihira Y, Tan CF, Hoshi Y, Iwanaga K, Yamada M, Kawachi I, Tsujihata M, Hozumi I, Morita T, Onodera O, Nishizawa M, Kakita A, Takahashi H
Acta neuropathologica 2009 Jan;117(1):45-53
Acta neuropathologica 2009 Jan;117(1):45-53
Progression of hippocampal degeneration in amyotrophic lateral sclerosis with or without memory impairment: distinction from Alzheimer disease.
Takeda T, Uchihara T, Arai N, Mizutani T, Iwata M
Acta neuropathologica 2009 Jan;117(1):35-44
Acta neuropathologica 2009 Jan;117(1):35-44
Wobbler mice modeling motor neuron disease display elevated transactive response DNA binding protein.
Dennis JS, Citron BA
Neuroscience 2009 Jan 23;158(2):745-50
Neuroscience 2009 Jan 23;158(2):745-50
Phosphorylated and ubiquitinated TDP-43 pathological inclusions in ALS and FTLD-U are recapitulated in SH-SY5Y cells.
Nonaka T, Arai T, Buratti E, Baralle FE, Akiyama H, Hasegawa M
FEBS letters 2009 Jan 22;583(2):394-400
FEBS letters 2009 Jan 22;583(2):394-400
Divergent patterns of cytosolic TDP-43 and neuronal progranulin expression following axotomy: implications for TDP-43 in the physiological response to neuronal injury.
Moisse K, Volkening K, Leystra-Lantz C, Welch I, Hill T, Strong MJ
Brain research 2009 Jan 16;1249:202-11
Brain research 2009 Jan 16;1249:202-11
Clinical and pathological characteristics of patients with leucine-rich repeat kinase-2 mutations.
Covy JP, Yuan W, Waxman EA, Hurtig HI, Van Deerlin VM, Giasson BI
Movement disorders : official journal of the Movement Disorder Society 2009 Jan 15;24(1):32-9
Movement disorders : official journal of the Movement Disorder Society 2009 Jan 15;24(1):32-9
Rapidly progressive neurodegenerative dementias.
Josephs KA, Ahlskog JE, Parisi JE, Boeve BF, Crum BA, Giannini C, Petersen RC
Archives of neurology 2009 Feb;66(2):201-7
Archives of neurology 2009 Feb;66(2):201-7
Clinical and pathological continuum of multisystem TDP-43 proteinopathies.
Geser F, Martinez-Lage M, Robinson J, Uryu K, Neumann M, Brandmeir NJ, Xie SX, Kwong LK, Elman L, McCluskey L, Clark CM, Malunda J, Miller BL, Zimmerman EA, Qian J, Van Deerlin V, Grossman M, Lee VM, Trojanowski JQ
Archives of neurology 2009 Feb;66(2):180-9
Archives of neurology 2009 Feb;66(2):180-9
TDP-43 accumulation is common in myopathies with rimmed vacuoles.
Küsters B, van Hoeve BJ, Schelhaas HJ, Ter Laak H, van Engelen BG, Lammens M
Acta neuropathologica 2009 Feb;117(2):209-11
Acta neuropathologica 2009 Feb;117(2):209-11
Accumulation of phosphorylated TDP-43 in brains of patients with argyrophilic grain disease.
Fujishiro H, Uchikado H, Arai T, Hasegawa M, Akiyama H, Yokota O, Tsuchiya K, Togo T, Iseki E, Hirayasu Y
Acta neuropathologica 2009 Feb;117(2):151-8
Acta neuropathologica 2009 Feb;117(2):151-8
Phosphorylation of S409/410 of TDP-43 is a consistent feature in all sporadic and familial forms of TDP-43 proteinopathies.
Neumann M, Kwong LK, Lee EB, Kremmer E, Flatley A, Xu Y, Forman MS, Troost D, Kretzschmar HA, Trojanowski JQ, Lee VM
Acta neuropathologica 2009 Feb;117(2):137-49
Acta neuropathologica 2009 Feb;117(2):137-49
Phosphorylated TDP-43 in Alzheimer's disease and dementia with Lewy bodies.
Arai T, Mackenzie IR, Hasegawa M, Nonoka T, Niizato K, Tsuchiya K, Iritani S, Onaya M, Akiyama H
Acta neuropathologica 2009 Feb;117(2):125-36
Acta neuropathologica 2009 Feb;117(2):125-36
Mutations in FUS, an RNA processing protein, cause familial amyotrophic lateral sclerosis type 6.
Vance C, Rogelj B, Hortobágyi T, De Vos KJ, Nishimura AL, Sreedharan J, Hu X, Smith B, Ruddy D, Wright P, Ganesalingam J, Williams KL, Tripathi V, Al-Saraj S, Al-Chalabi A, Leigh PN, Blair IP, Nicholson G, de Belleroche J, Gallo JM, Miller CC, Shaw CE
Science (New York, N.Y.) 2009 Feb 27;323(5918):1208-1211
Science (New York, N.Y.) 2009 Feb 27;323(5918):1208-1211
Absence of Pittsburgh compound B detection of cerebral amyloid beta in a patient with clinical, cognitive, and cerebrospinal fluid markers of Alzheimer disease: a case report.
Cairns NJ, Ikonomovic MD, Benzinger T, Storandt M, Fagan AM, Shah AR, Reinwald LT, Carter D, Felton A, Holtzman DM, Mintun MA, Klunk WE, Morris JC
Archives of neurology 2009 Dec;66(12):1557-62
Archives of neurology 2009 Dec;66(12):1557-62
Transactive response DNA-binding protein 43 burden in familial Alzheimer disease and Down syndrome.
Lippa CF, Rosso AL, Stutzbach LD, Neumann M, Lee VM, Trojanowski JQ
Archives of neurology 2009 Dec;66(12):1483-8
Archives of neurology 2009 Dec;66(12):1483-8
Sporadic amyotrophic lateral sclerosis: Widespread multisystem degeneration with TDP-43 pathology in a patient after long-term survival on a respirator.
Nishihira Y, Tan CF, Toyoshima Y, Yonemochi Y, Kondo H, Nakajima T, Takahashi H
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Dec;29(6):689-96
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Dec;29(6):689-96
TDP-43 is consistently co-localized with ubiquitinated inclusions in sporadic and Guam amyotrophic lateral sclerosis but not in familial amyotrophic lateral sclerosis with and without SOD1 mutations.
Maekawa S, Leigh PN, King A, Jones E, Steele JC, Bodi I, Shaw CE, Hortobagyi T, Al-Sarraj S
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Dec;29(6):672-83
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Dec;29(6):672-83
Axonal ligation induces transient redistribution of TDP-43 in brainstem motor neurons.
Sato T, Takeuchi S, Saito A, Ding W, Bamba H, Matsuura H, Hisa Y, Tooyama I, Urushitani M
Neuroscience 2009 Dec 29;164(4):1565-78
Neuroscience 2009 Dec 29;164(4):1565-78
Valosin-containing protein (VCP) is required for autophagy and is disrupted in VCP disease.
Ju JS, Fuentealba RA, Miller SE, Jackson E, Piwnica-Worms D, Baloh RH, Weihl CC
The Journal of cell biology 2009 Dec 14;187(6):875-88
The Journal of cell biology 2009 Dec 14;187(6):875-88
Tar DNA binding protein of 43 kDa (TDP-43), 14-3-3 proteins and copper/zinc superoxide dismutase (SOD1) interact to modulate NFL mRNA stability. Implications for altered RNA processing in amyotrophic lateral sclerosis (ALS).
Volkening K, Leystra-Lantz C, Yang W, Jaffee H, Strong MJ
Brain research 2009 Dec 11;1305:168-82
Brain research 2009 Dec 11;1305:168-82
The dorsal raphe nucleus shows phospho-tau neurofibrillary changes before the transentorhinal region in Alzheimer's disease. A precocious onset?
Grinberg LT, Rüb U, Ferretti RE, Nitrini R, Farfel JM, Polichiso L, Gierga K, Jacob-Filho W, Heinsen H, Brazilian Brain Bank Study Group
Neuropathology and applied neurobiology 2009 Aug;35(4):406-16
Neuropathology and applied neurobiology 2009 Aug;35(4):406-16
Frontotemporal lobar degeneration with ubiquitinated tau-negative inclusions and additional alpha-synuclein pathology but also unusual cerebellar ubiquitinated p62-positive, TDP-43-negative inclusions.
King A, Al-Sarraj S, Shaw C
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Aug;29(4):466-71
Neuropathology : official journal of the Japanese Society of Neuropathology 2009 Aug;29(4):466-71
TDP-43 pathology in familial British dementia.
Schwab C, Arai T, Hasegawa M, Akiyama H, Yu S, McGeer PL
Acta neuropathologica 2009 Aug;118(2):303-11
Acta neuropathologica 2009 Aug;118(2):303-11
Proteolytic processing of TAR DNA binding protein-43 by caspases produces C-terminal fragments with disease defining properties independent of progranulin.
Dormann D, Capell A, Carlson AM, Shankaran SS, Rodde R, Neumann M, Kremmer E, Matsuwaki T, Yamanouchi K, Nishihara M, Haass C
Journal of neurochemistry 2009 Aug;110(3):1082-94
Journal of neurochemistry 2009 Aug;110(3):1082-94
Clinical heterogeneity in 3 unrelated families linked to VCP p.Arg159His.
van der Zee J, Pirici D, Van Langenhove T, Engelborghs S, Vandenberghe R, Hoffmann M, Pusswald G, Van den Broeck M, Peeters K, Mattheijssens M, Martin JJ, De Deyn PP, Cruts M, Haubenberger D, Kumar-Singh S, Zimprich A, Van Broeckhoven C
Neurology 2009 Aug 25;73(8):626-32
Neurology 2009 Aug 25;73(8):626-32
TDP-43 depletion induces neuronal cell damage through dysregulation of Rho family GTPases.
Iguchi Y, Katsuno M, Niwa JI, Yamada SI, Sone J, Waza M, Adachi H, Tanaka F, Nagata KI, Arimura N, Watanabe T, Kaibuchi K, Sobue G
The Journal of biological chemistry 2009 Aug 14;284(33):22059-22066
The Journal of biological chemistry 2009 Aug 14;284(33):22059-22066
TDP-43 neuropathology is similar in sporadic amyotrophic lateral sclerosis with or without TDP-43 mutations.
Pamphlett R, Luquin N, McLean C, Jew SK, Adams L
Neuropathology and applied neurobiology 2009 Apr;35(2):222-5
Neuropathology and applied neurobiology 2009 Apr;35(2):222-5
Ubiquitin associated protein 1 is a risk factor for frontotemporal lobar degeneration.
Rollinson S, Rizzu P, Sikkink S, Baker M, Halliwell N, Snowden J, Traynor BJ, Ruano D, Cairns N, Rohrer JD, Mead S, Collinge J, Rossor M, Akay E, Guerreiro R, Rademakers R, Morrison KE, Pastor P, Alonso E, Martinez-Lage P, Graff-Radford N, Neary D, Heutink P, Mann DM, Van Swieten J, Pickering-Brown SM
Neurobiology of aging 2009 Apr;30(4):656-65
Neurobiology of aging 2009 Apr;30(4):656-65
Mimicking aspects of frontotemporal lobar degeneration and Lou Gehrig's disease in rats via TDP-43 overexpression.
Tatom JB, Wang DB, Dayton RD, Skalli O, Hutton ML, Dickson DW, Klein RL
Molecular therapy : the journal of the American Society of Gene Therapy 2009 Apr;17(4):607-13
Molecular therapy : the journal of the American Society of Gene Therapy 2009 Apr;17(4):607-13
Neuropathologic classification of dementias: introduction.
Duyckaerts C
Handbook of clinical neurology 2008;89:147-59
Handbook of clinical neurology 2008;89:147-59
Frontotemporal dementia mimicking dementia with Lewy bodies.
Claassen DO, Parisi JE, Giannini C, Boeve BF, Dickson DW, Josephs KA
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology 2008 Sep;21(3):157-63
Cognitive and behavioral neurology : official journal of the Society for Behavioral and Cognitive Neurology 2008 Sep;21(3):157-63
Caspase-cleaved TAR DNA-binding protein-43 is a major pathological finding in Alzheimer's disease.
Rohn TT
Brain research 2008 Sep 4;1228:189-98
Brain research 2008 Sep 4;1228:189-98
Novel mutations in TARDBP (TDP-43) in patients with familial amyotrophic lateral sclerosis.
Rutherford NJ, Zhang YJ, Baker M, Gass JM, Finch NA, Xu YF, Stewart H, Kelley BJ, Kuntz K, Crook RJ, Sreedharan J, Vance C, Sorenson E, Lippa C, Bigio EH, Geschwind DH, Knopman DS, Mitsumoto H, Petersen RC, Cashman NR, Hutton M, Shaw CE, Boylan KB, Boeve B, Graff-Radford NR, Wszolek ZK, Caselli RJ, Dickson DW, Mackenzie IR, Petrucelli L, Rademakers R
PLoS genetics 2008 Sep 19;4(9):e1000193
PLoS genetics 2008 Sep 19;4(9):e1000193
TDP-43 accumulation in inclusion body myopathy muscle suggests a common pathogenic mechanism with frontotemporal dementia.
Weihl CC, Temiz P, Miller SE, Watts G, Smith C, Forman M, Hanson PI, Kimonis V, Pestronk A
Journal of neurology, neurosurgery, and psychiatry 2008 Oct;79(10):1186-9
Journal of neurology, neurosurgery, and psychiatry 2008 Oct;79(10):1186-9
White matter tauopathy with globular glial inclusions: a distinct sporadic frontotemporal lobar degeneration.
Kovacs GG, Majtenyi K, Spina S, Murrell JR, Gelpi E, Hoftberger R, Fraser G, Crowther RA, Goedert M, Budka H, Ghetti B
Journal of neuropathology and experimental neurology 2008 Oct;67(10):963-75
Journal of neuropathology and experimental neurology 2008 Oct;67(10):963-75
Immunohistochemical identification of messenger RNA-related proteins in basophilic inclusions of adult-onset atypical motor neuron disease.
Fujita K, Ito H, Nakano S, Kinoshita Y, Wate R, Kusaka H
Acta neuropathologica 2008 Oct;116(4):439-45
Acta neuropathologica 2008 Oct;116(4):439-45
TDP-43 expression in mouse models of amyotrophic lateral sclerosis and spinal muscular atrophy.
Turner BJ, Bäumer D, Parkinson NJ, Scaber J, Ansorge O, Talbot K
BMC neuroscience 2008 Oct 28;9:104
BMC neuroscience 2008 Oct 28;9:104
TDP-43 in cerebrospinal fluid of patients with frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Steinacker P, Hendrich C, Sperfeld AD, Jesse S, von Arnim CA, Lehnert S, Pabst A, Uttner I, Tumani H, Lee VM, Trojanowski JQ, Kretzschmar HA, Ludolph A, Neumann M, Otto M
Archives of neurology 2008 Nov;65(11):1481-7
Archives of neurology 2008 Nov;65(11):1481-7
Progranulin mutation causes frontotemporal dementia in the Swedish Karolinska family.
Chiang HH, Rosvall L, Brohede J, Axelman K, Björk BF, Nennesmo I, Robins T, Graff C
Alzheimer's & dementia : the journal of the Alzheimer's Association 2008 Nov;4(6):414-20
Alzheimer's & dementia : the journal of the Alzheimer's Association 2008 Nov;4(6):414-20
Structural determinants of the cellular localization and shuttling of TDP-43.
Ayala YM, Zago P, D'Ambrogio A, Xu YF, Petrucelli L, Buratti E, Baralle FE
Journal of cell science 2008 Nov 15;121(Pt 22):3778-85
Journal of cell science 2008 Nov 15;121(Pt 22):3778-85
Evidence of multisystem disorder in whole-brain map of pathological TDP-43 in amyotrophic lateral sclerosis.
Geser F, Brandmeir NJ, Kwong LK, Martinez-Lage M, Elman L, McCluskey L, Xie SX, Lee VM, Trojanowski JQ
Archives of neurology 2008 May;65(5):636-41
Archives of neurology 2008 May;65(5):636-41
Pilot study of granulocyte colony stimulating factor (G-CSF)-mobilized peripheral blood stem cells in amyotrophic lateral sclerosis (ALS).
Cashman N, Tan LY, Krieger C, Mädler B, Mackay A, Mackenzie I, Benny B, Nantel S, Fabros M, Shinobu L, Yousefi M, Eisen A
Muscle & nerve 2008 May;37(5):620-5
Muscle & nerve 2008 May;37(5):620-5
Atypical frontotemporal lobar degeneration with ubiquitin-positive, TDP-43-negative neuronal inclusions.
Mackenzie IR, Foti D, Woulfe J, Hurwitz TA
Brain : a journal of neurology 2008 May;131(Pt 5):1282-93
Brain : a journal of neurology 2008 May;131(Pt 5):1282-93
Basophilic inclusion body disease and neuronal intermediate filament inclusion disease: a comparative clinicopathological study.
Yokota O, Tsuchiya K, Terada S, Ishizu H, Uchikado H, Ikeda M, Oyanagi K, Nakano I, Murayama S, Kuroda S, Akiyama H
Acta neuropathologica 2008 May;115(5):561-75
Acta neuropathologica 2008 May;115(5):561-75
Disturbance of nuclear and cytoplasmic TAR DNA-binding protein (TDP-43) induces disease-like redistribution, sequestration, and aggregate formation.
Winton MJ, Igaz LM, Wong MM, Kwong LK, Trojanowski JQ, Lee VM
The Journal of biological chemistry 2008 May 9;283(19):13302-9
The Journal of biological chemistry 2008 May 9;283(19):13302-9
Abnormal TDP-43 immunoreactivity in AD modifies clinicopathologic and radiologic phenotype.
Josephs KA, Whitwell JL, Knopman DS, Hu WT, Stroh DA, Baker M, Rademakers R, Boeve BF, Parisi JE, Smith GE, Ivnik RJ, Petersen RC, Jack CR Jr, Dickson DW
Neurology 2008 May 6;70(19 Pt 2):1850-7
Neurology 2008 May 6;70(19 Pt 2):1850-7
Frequency and clinical characteristics of progranulin mutation carriers in the Manchester frontotemporal lobar degeneration cohort: comparison with patients with MAPT and no known mutations.
Pickering-Brown SM, Rollinson S, Du Plessis D, Morrison KE, Varma A, Richardson AM, Neary D, Snowden JS, Mann DM
Brain : a journal of neurology 2008 Mar;131(Pt 3):721-31
Brain : a journal of neurology 2008 Mar;131(Pt 3):721-31
Phosphorylated Smad2/3 immunoreactivity in sporadic and familial amyotrophic lateral sclerosis and its mouse model.
Nakamura M, Ito H, Wate R, Nakano S, Hirano A, Kusaka H
Acta neuropathologica 2008 Mar;115(3):327-34
Acta neuropathologica 2008 Mar;115(3):327-34
TDP-43 immunoreactivity in anoxic, ischemic and neoplastic lesions of the central nervous system.
Lee EB, Lee VM, Trojanowski JQ, Neumann M
Acta neuropathologica 2008 Mar;115(3):305-11
Acta neuropathologica 2008 Mar;115(3):305-11
Epitope mapping of 2E2-D3, a monoclonal antibody directed against human TDP-43.
Zhang HX, Tanji K, Mori F, Wakabayashi K
Neuroscience letters 2008 Mar 28;434(2):170-4
Neuroscience letters 2008 Mar 28;434(2):170-4
TDP-43 mutations in familial and sporadic amyotrophic lateral sclerosis.
Sreedharan J, Blair IP, Tripathi VB, Hu X, Vance C, Rogelj B, Ackerley S, Durnall JC, Williams KL, Buratti E, Baralle F, de Belleroche J, Mitchell JD, Leigh PN, Al-Chalabi A, Miller CC, Nicholson G, Shaw CE
Science (New York, N.Y.) 2008 Mar 21;319(5870):1668-72
Science (New York, N.Y.) 2008 Mar 21;319(5870):1668-72
Concomitant TAR-DNA-binding protein 43 pathology is present in Alzheimer disease and corticobasal degeneration but not in other tauopathies.
Uryu K, Nakashima-Yasuda H, Forman MS, Kwong LK, Clark CM, Grossman M, Miller BL, Kretzschmar HA, Lee VM, Trojanowski JQ, Neumann M
Journal of neuropathology and experimental neurology 2008 Jun;67(6):555-64
Journal of neuropathology and experimental neurology 2008 Jun;67(6):555-64
Alzheimer and frontotemporal pathology in subsets of primary progressive aphasia.
Mesulam M, Wicklund A, Johnson N, Rogalski E, Léger GC, Rademaker A, Weintraub S, Bigio EH
Annals of neurology 2008 Jun;63(6):709-19
Annals of neurology 2008 Jun;63(6):709-19
A90V TDP-43 variant results in the aberrant localization of TDP-43 in vitro.
Winton MJ, Van Deerlin VM, Kwong LK, Yuan W, Wood EM, Yu CE, Schellenberg GD, Rademakers R, Caselli R, Karydas A, Trojanowski JQ, Miller BL, Lee VM
FEBS letters 2008 Jun 25;582(15):2252-6
FEBS letters 2008 Jun 25;582(15):2252-6
Anterior horn cells with abnormal TDP-43 immunoreactivities show fragmentation of the Golgi apparatus in ALS.
Fujita Y, Mizuno Y, Takatama M, Okamoto K
Journal of the neurological sciences 2008 Jun 15;269(1-2):30-4
Journal of the neurological sciences 2008 Jun 15;269(1-2):30-4
Progranulin gene mutation with an unusual clinical and neuropathologic presentation.
Wider C, Uitti RJ, Wszolek ZK, Fang JY, Josephs KA, Baker MC, Rademakers R, Hutton ML, Dickson DW
Movement disorders : official journal of the Movement Disorder Society 2008 Jun 15;23(8):1168-73
Movement disorders : official journal of the Movement Disorder Society 2008 Jun 15;23(8):1168-73
Update on recent molecular and genetic advances in frontotemporal lobar degeneration.
Bigio EH
Journal of neuropathology and experimental neurology 2008 Jul;67(7):635-48
Journal of neuropathology and experimental neurology 2008 Jul;67(7):635-48
Phosphorylated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Hasegawa M, Arai T, Nonaka T, Kametani F, Yoshida M, Hashizume Y, Beach TG, Buratti E, Baralle F, Morita M, Nakano I, Oda T, Tsuchiya K, Akiyama H
Annals of neurology 2008 Jul;64(1):60-70
Annals of neurology 2008 Jul;64(1):60-70
Enrichment of C-terminal fragments in TAR DNA-binding protein-43 cytoplasmic inclusions in brain but not in spinal cord of frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Igaz LM, Kwong LK, Xu Y, Truax AC, Uryu K, Neumann M, Clark CM, Elman LB, Miller BL, Grossman M, McCluskey LF, Trojanowski JQ, Lee VM
The American journal of pathology 2008 Jul;173(1):182-94
The American journal of pathology 2008 Jul;173(1):182-94
Clinical, neuropathological and genotypic variability in SNCA A53T familial Parkinson's disease. Variability in familial Parkinson's disease.
Markopoulou K, Dickson DW, McComb RD, Wszolek ZK, Katechalidou L, Avery L, Stansbury MS, Chase BA
Acta neuropathologica 2008 Jul;116(1):25-35
Acta neuropathologica 2008 Jul;116(1):25-35
Early onset familial Alzheimer Disease with spastic paraparesis, dysarthria, and seizures and N135S mutation in PSEN1.
Rudzinski LA, Fletcher RM, Dickson DW, Crook R, Hutton ML, Adamson J, Graff-Radford NR
Alzheimer disease and associated disorders 2008 Jul-Sep;22(3):299-307
Alzheimer disease and associated disorders 2008 Jul-Sep;22(3):299-307
TAR-DNA binding protein 43 in Pick disease.
Freeman SH, Spires-Jones T, Hyman BT, Growdon JH, Frosch MP
Journal of neuropathology and experimental neurology 2008 Jan;67(1):62-7
Journal of neuropathology and experimental neurology 2008 Jan;67(1):62-7
Pathological TDP-43 in parkinsonism-dementia complex and amyotrophic lateral sclerosis of Guam.
Geser F, Winton MJ, Kwong LK, Xu Y, Xie SX, Igaz LM, Garruto RM, Perl DP, Galasko D, Lee VM, Trojanowski JQ
Acta neuropathologica 2008 Jan;115(1):133-45
Acta neuropathologica 2008 Jan;115(1):133-45
Severe subcortical TDP-43 pathology in sporadic frontotemporal lobar degeneration with motor neuron disease.
Brandmeir NJ, Geser F, Kwong LK, Zimmerman E, Qian J, Lee VM, Trojanowski JQ
Acta neuropathologica 2008 Jan;115(1):123-31
Acta neuropathologica 2008 Jan;115(1):123-31
TDP-43-immunoreactive neuronal and glial inclusions in the neostriatum in amyotrophic lateral sclerosis with and without dementia.
Zhang H, Tan CF, Mori F, Tanji K, Kakita A, Takahashi H, Wakabayashi K
Acta neuropathologica 2008 Jan;115(1):115-22
Acta neuropathologica 2008 Jan;115(1):115-22
Missense mutations in the progranulin gene linked to frontotemporal lobar degeneration with ubiquitin-immunoreactive inclusions reduce progranulin production and secretion.
Shankaran SS, Capell A, Hruscha AT, Fellerer K, Neumann M, Schmid B, Haass C
The Journal of biological chemistry 2008 Jan 18;283(3):1744-1753
The Journal of biological chemistry 2008 Jan 18;283(3):1744-1753
Liver X receptor beta (LXRbeta): a link between beta-sitosterol and amyotrophic lateral sclerosis-Parkinson's dementia.
Kim HJ, Fan X, Gabbi C, Yakimchuk K, Parini P, Warner M, Gustafsson JA
Proceedings of the National Academy of Sciences of the United States of America 2008 Feb 12;105(6):2094-9
Proceedings of the National Academy of Sciences of the United States of America 2008 Feb 12;105(6):2094-9
Colocalization of transactivation-responsive DNA-binding protein 43 and huntingtin in inclusions of Huntington disease.
Schwab C, Arai T, Hasegawa M, Yu S, McGeer PL
Journal of neuropathology and experimental neurology 2008 Dec;67(12):1159-65
Journal of neuropathology and experimental neurology 2008 Dec;67(12):1159-65
LRRK2 and parkin immunoreactivity in multiple system atrophy inclusions.
Huang Y, Song YJ, Murphy K, Holton JL, Lashley T, Revesz T, Gai WP, Halliday GM
Acta neuropathologica 2008 Dec;116(6):639-46
Acta neuropathologica 2008 Dec;116(6):639-46
Enduring involvement of tau, beta-amyloid, alpha-synuclein, ubiquitin and TDP-43 pathology in the amyotrophic lateral sclerosis/parkinsonism-dementia complex of Guam (ALS/PDC).
Miklossy J, Steele JC, Yu S, McCall S, Sandberg G, McGeer EG, McGeer PL
Acta neuropathologica 2008 Dec;116(6):625-37
Acta neuropathologica 2008 Dec;116(6):625-37
Fine structural analysis of the neuronal inclusions of frontotemporal lobar degeneration with TDP-43 proteinopathy.
Thorpe JR, Tang H, Atherton J, Cairns NJ
Journal of neural transmission (Vienna, Austria : 1996) 2008 Dec;115(12):1661-71
Journal of neural transmission (Vienna, Austria : 1996) 2008 Dec;115(12):1661-71
Common variation in the miR-659 binding-site of GRN is a major risk factor for TDP43-positive frontotemporal dementia.
Rademakers R, Eriksen JL, Baker M, Robinson T, Ahmed Z, Lincoln SJ, Finch N, Rutherford NJ, Crook RJ, Josephs KA, Boeve BF, Knopman DS, Petersen RC, Parisi JE, Caselli RJ, Wszolek ZK, Uitti RJ, Feldman H, Hutton ML, Mackenzie IR, Graff-Radford NR, Dickson DW
Human molecular genetics 2008 Dec 1;17(23):3631-42
Human molecular genetics 2008 Dec 1;17(23):3631-42
Lack of TAR-DNA binding protein-43 (TDP-43) pathology in human prion diseases.
Isaacs AM, Powell C, Webb TE, Linehan JM, Collinge J, Brandner S
Neuropathology and applied neurobiology 2008 Aug;34(4):446-56
Neuropathology and applied neurobiology 2008 Aug;34(4):446-56
TDP-43 inclusions do not protect motor neurons from sporadic ALS.
Pamphlett R, Kum Jew S
Acta neuropathologica 2008 Aug;116(2):221-2
Acta neuropathologica 2008 Aug;116(2):221-2
Temporal lobar predominance of TDP-43 neuronal cytoplasmic inclusions in Alzheimer disease.
Hu WT, Josephs KA, Knopman DS, Boeve BF, Dickson DW, Petersen RC, Parisi JE
Acta neuropathologica 2008 Aug;116(2):215-20
Acta neuropathologica 2008 Aug;116(2):215-20
Ultrastructural localization of TDP-43 in filamentous neuronal inclusions in various neurodegenerative diseases.
Lin WL, Dickson DW
Acta neuropathologica 2008 Aug;116(2):205-13
Acta neuropathologica 2008 Aug;116(2):205-13
Maturation process of TDP-43-positive neuronal cytoplasmic inclusions in amyotrophic lateral sclerosis with and without dementia.
Mori F, Tanji K, Zhang HX, Nishihira Y, Tan CF, Takahashi H, Wakabayashi K
Acta neuropathologica 2008 Aug;116(2):193-203
Acta neuropathologica 2008 Aug;116(2):193-203
Sporadic amyotrophic lateral sclerosis: two pathological patterns shown by analysis of distribution of TDP-43-immunoreactive neuronal and glial cytoplasmic inclusions.
Nishihira Y, Tan CF, Onodera O, Toyoshima Y, Yamada M, Morita T, Nishizawa M, Kakita A, Takahashi H
Acta neuropathologica 2008 Aug;116(2):169-82
Acta neuropathologica 2008 Aug;116(2):169-82
Frontotemporal lobar degeneration with ubiquitin-positive, but TDP-43-negative inclusions.
Josephs KA, Lin WL, Ahmed Z, Stroh DA, Graff-Radford NR, Dickson DW
Acta neuropathologica 2008 Aug;116(2):159-67
Acta neuropathologica 2008 Aug;116(2):159-67
TDP-43-negative FTLD-U is a significant new clinico-pathological subtype of FTLD.
Roeber S, Mackenzie IR, Kretzschmar HA, Neumann M
Acta neuropathologica 2008 Aug;116(2):147-57
Acta neuropathologica 2008 Aug;116(2):147-57
TDP-43 protein in plasma may index TDP-43 brain pathology in Alzheimer's disease and frontotemporal lobar degeneration.
Foulds P, McAuley E, Gibbons L, Davidson Y, Pickering-Brown SM, Neary D, Snowden JS, Allsop D, Mann DM
Acta neuropathologica 2008 Aug;116(2):141-6
Acta neuropathologica 2008 Aug;116(2):141-6
Pedigree with frontotemporal lobar degeneration--motor neuron disease and Tar DNA binding protein-43 positive neuropathology: genetic linkage to chromosome 9.
Luty AA, Kwok JB, Thompson EM, Blumbergs P, Brooks WS, Loy CT, Dobson-Stone C, Panegyres PK, Hecker J, Nicholson GA, Halliday GM, Schofield PR
BMC neurology 2008 Aug 29;8:32
BMC neurology 2008 Aug 29;8:32
Abnormal phosphorylation of Ser409/410 of TDP-43 in FTLD-U and ALS.
Inukai Y, Nonaka T, Arai T, Yoshida M, Hashizume Y, Beach TG, Buratti E, Baralle FE, Akiyama H, Hisanaga S, Hasegawa M
FEBS letters 2008 Aug 20;582(19):2899-904
FEBS letters 2008 Aug 20;582(19):2899-904
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions visualized with ubiquitin-binding protein p62 immunohistochemistry.
Pikkarainen M, Hartikainen P, Alafuzoff I
Journal of neuropathology and experimental neurology 2008 Apr;67(4):280-98
Journal of neuropathology and experimental neurology 2008 Apr;67(4):280-98
TAR DNA-binding protein 43 immunohistochemistry reveals extensive neuritic pathology in FTLD-U: a midwest-southwest consortium for FTLD study.
Hatanpaa KJ, Bigio EH, Cairns NJ, Womack KB, Weintraub S, Morris JC, Foong C, Xiao G, Hladik C, Mantanona TY, White CL 3rd
Journal of neuropathology and experimental neurology 2008 Apr;67(4):271-9
Journal of neuropathology and experimental neurology 2008 Apr;67(4):271-9
TDP-43 mutation in familial amyotrophic lateral sclerosis.
Yokoseki A, Shiga A, Tan CF, Tagawa A, Kaneko H, Koyama A, Eguchi H, Tsujino A, Ikeuchi T, Kakita A, Okamoto K, Nishizawa M, Takahashi H, Onodera O
Annals of neurology 2008 Apr;63(4):538-42
Annals of neurology 2008 Apr;63(4):538-42
Co-morbidity of TDP-43 proteinopathy in Lewy body related diseases.
Nakashima-Yasuda H, Uryu K, Robinson J, Xie SX, Hurtig H, Duda JE, Arnold SE, Siderowf A, Grossman M, Leverenz JB, Woltjer R, Lopez OL, Hamilton R, Tsuang DW, Galasko D, Masliah E, Kaye J, Clark CM, Montine TJ, Lee VM, Trojanowski JQ
Acta neuropathologica 2007 Sep;114(3):221-9
Acta neuropathologica 2007 Sep;114(3):221-9
Progranulin mediates caspase-dependent cleavage of TAR DNA binding protein-43.
Zhang YJ, Xu YF, Dickey CA, Buratti E, Baralle F, Bailey R, Pickering-Brown S, Dickson D, Petrucelli L
The Journal of neuroscience : the official journal of the Society for Neuroscience 2007 Sep 26;27(39):10530-4
The Journal of neuroscience : the official journal of the Society for Neuroscience 2007 Sep 26;27(39):10530-4
Corticobasal syndrome associated with the A9D Progranulin mutation.
Spina S, Murrell JR, Huey ED, Wassermann EM, Pietrini P, Grafman J, Ghetti B
Journal of neuropathology and experimental neurology 2007 Oct;66(10):892-900
Journal of neuropathology and experimental neurology 2007 Oct;66(10):892-900
A reassessment of the neuropathology of frontotemporal dementia linked to chromosome 3.
Holm IE, Englund E, Mackenzie IR, Johannsen P, Isaacs AM
Journal of neuropathology and experimental neurology 2007 Oct;66(10):884-91
Journal of neuropathology and experimental neurology 2007 Oct;66(10):884-91
Alzheimer and Parkinson diagnoses in progranulin null mutation carriers in an extended founder family.
Brouwers N, Nuytemans K, van der Zee J, Gijselinck I, Engelborghs S, Theuns J, Kumar-Singh S, Pickut BA, Pals P, Dermaut B, Bogaerts V, De Pooter T, Serneels S, Van den Broeck M, Cuijt I, Mattheijssens M, Peeters K, Sciot R, Martin JJ, Cras P, Santens P, Vandenberghe R, De Deyn PP, Cruts M, Van Broeckhoven C, Sleegers K
Archives of neurology 2007 Oct;64(10):1436-46
Archives of neurology 2007 Oct;64(10):1436-46
Progressive anomia revisited: focal degeneration associated with progranulin gene mutation.
Snowden JS, Pickering-Brown SM, Du Plessis D, Mackenzie IR, Varma A, Mann DM, Neary D
Neurocase 2007 Oct;13(5):366-77
Neurocase 2007 Oct;13(5):366-77
Frontotemporal lobar degeneration with upper motor neuron disease/ primary lateral sclerosis.
Josephs KA, Dickson DW
Neurology 2007 Oct 30;69(18):1800-1
Neurology 2007 Oct 30;69(18):1800-1
Increase in the relative expression of tau with four microtubule binding repeat regions in frontotemporal lobar degeneration and progressive supranuclear palsy brains.
Ingelsson M, Ramasamy K, Russ C, Freeman SH, Orne J, Raju S, Matsui T, Growdon JH, Frosch MP, Ghetti B, Brown RH, Irizarry MC, Hyman BT
Acta neuropathologica 2007 Nov;114(5):471-9
Acta neuropathologica 2007 Nov;114(5):471-9
Functional multivesicular bodies are required for autophagic clearance of protein aggregates associated with neurodegenerative disease.
Filimonenko M, Stuffers S, Raiborg C, Yamamoto A, Malerød L, Fisher EM, Isaacs A, Brech A, Stenmark H, Simonsen A
The Journal of cell biology 2007 Nov 5;179(3):485-500
The Journal of cell biology 2007 Nov 5;179(3):485-500
TDP-43 immunoreactivity in hippocampal sclerosis and Alzheimer's disease.
Amador-Ortiz C, Lin WL, Ahmed Z, Personett D, Davies P, Duara R, Graff-Radford NR, Hutton ML, Dickson DW
Annals of neurology 2007 May;61(5):435-45
Annals of neurology 2007 May;61(5):435-45
Pathological TDP-43 distinguishes sporadic amyotrophic lateral sclerosis from amyotrophic lateral sclerosis with SOD1 mutations.
Mackenzie IR, Bigio EH, Ince PG, Geser F, Neumann M, Cairns NJ, Kwong LK, Forman MS, Ravits J, Stewart H, Eisen A, McClusky L, Kretzschmar HA, Monoranu CM, Highley JR, Kirby J, Siddique T, Shaw PJ, Lee VM, Trojanowski JQ
Annals of neurology 2007 May;61(5):427-34
Annals of neurology 2007 May;61(5):427-34
TDP-43 is deposited in the Guam parkinsonism-dementia complex brains.
Hasegawa M, Arai T, Akiyama H, Nonaka T, Mori H, Hashimoto T, Yamazaki M, Oyanagi K
Brain : a journal of neurology 2007 May;130(Pt 5):1386-94
Brain : a journal of neurology 2007 May;130(Pt 5):1386-94
A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology.
Leverenz JB, Yu CE, Montine TJ, Steinbart E, Bekris LM, Zabetian C, Kwong LK, Lee VM, Schellenberg GD, Bird TD
Brain : a journal of neurology 2007 May;130(Pt 5):1360-74
Brain : a journal of neurology 2007 May;130(Pt 5):1360-74
Absence of heterogeneous nuclear ribonucleoproteins and survival motor neuron protein in TDP-43 positive inclusions in frontotemporal lobar degeneration.
Neumann M, Igaz LM, Kwong LK, Nakashima-Yasuda H, Kolb SJ, Dreyfuss G, Kretzschmar HA, Trojanowski JQ, Lee VM
Acta neuropathologica 2007 May;113(5):543-8
Acta neuropathologica 2007 May;113(5):543-8
TDP-43 immunoreactivity in neuronal inclusions in familial amyotrophic lateral sclerosis with or without SOD1 gene mutation.
Tan CF, Eguchi H, Tagawa A, Onodera O, Iwasaki T, Tsujino A, Nishizawa M, Kakita A, Takahashi H
Acta neuropathologica 2007 May;113(5):535-42
Acta neuropathologica 2007 May;113(5):535-42
Ubiquitinated pathological lesions in frontotemporal lobar degeneration contain the TAR DNA-binding protein, TDP-43.
Davidson Y, Kelley T, Mackenzie IR, Pickering-Brown S, Du Plessis D, Neary D, Snowden JS, Mann DM
Acta neuropathologica 2007 May;113(5):521-33
Acta neuropathologica 2007 May;113(5):521-33
TDP-43-positive white matter pathology in frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Neumann M, Kwong LK, Truax AC, Vanmassenhove B, Kretzschmar HA, Van Deerlin VM, Clark CM, Grossman M, Miller BL, Trojanowski JQ, Lee VM
Journal of neuropathology and experimental neurology 2007 Mar;66(3):177-83
Journal of neuropathology and experimental neurology 2007 Mar;66(3):177-83
Clinicopathologic features of frontotemporal dementia with progranulin sequence variation.
Spina S, Murrell JR, Huey ED, Wassermann EM, Pietrini P, Baraibar MA, Barbeito AG, Troncoso JC, Vidal R, Ghetti B, Grafman J
Neurology 2007 Mar 13;68(11):820-7
Neurology 2007 Mar 13;68(11):820-7
Nuclear protein profiling of Jurkat cells during heat stress-induced apoptosis by 2-DE and MS/MS.
Yuan X, Kuramitsu Y, Furumoto H, Zhang X, Hayashi E, Fujimoto M, Nakamura K
Electrophoresis 2007 Jun;28(12):2018-26
Electrophoresis 2007 Jun;28(12):2018-26
Appearance pattern of TDP-43 in Japanese frontotemporal lobar degeneration with ubiquitin-positive inclusions.
Higashi S, Iseki E, Yamamoto R, Minegishi M, Hino H, Fujisawa K, Togo T, Katsuse O, Uchikado H, Furukawa Y, Kosaka K, Arai H
Neuroscience letters 2007 Jun 4;419(3):213-8
Neuroscience letters 2007 Jun 4;419(3):213-8
Lack of TDP-43 abnormalities in mutant SOD1 transgenic mice shows disparity with ALS.
Robertson J, Sanelli T, Xiao S, Yang W, Horne P, Hammond R, Pioro EP, Strong MJ
Neuroscience letters 2007 Jun 13;420(2):128-32
Neuroscience letters 2007 Jun 13;420(2):128-32
TDP-43 in familial and sporadic frontotemporal lobar degeneration with ubiquitin inclusions.
Cairns NJ, Neumann M, Bigio EH, Holm IE, Troost D, Hatanpaa KJ, Foong C, White CL 3rd, Schneider JA, Kretzschmar HA, Carter D, Taylor-Reinwald L, Paulsmeyer K, Strider J, Gitcho M, Goate AM, Morris JC, Mishra M, Kwong LK, Stieber A, Xu Y, Forman MS, Trojanowski JQ, Lee VM, Mackenzie IR
The American journal of pathology 2007 Jul;171(1):227-40
The American journal of pathology 2007 Jul;171(1):227-40
TDP-43 in differential diagnosis of motor neuron disorders.
Dickson DW, Josephs KA, Amador-Ortiz C
Acta neuropathologica 2007 Jul;114(1):71-9
Acta neuropathologica 2007 Jul;114(1):71-9
A 34-year-old man with progressive behavioral and language disturbance.
Miller BL, Josephs KA Jr, Parisi JE, Keegan BM
Neurology 2007 Jan 2;68(1):68-74
Neurology 2007 Jan 2;68(1):68-74
TDP-43 in the ubiquitin pathology of frontotemporal dementia with VCP gene mutations.
Neumann M, Mackenzie IR, Cairns NJ, Boyer PJ, Markesbery WR, Smith CD, Taylor JP, Kretzschmar HA, Kimonis VE, Forman MS
Journal of neuropathology and experimental neurology 2007 Feb;66(2):152-7
Journal of neuropathology and experimental neurology 2007 Feb;66(2):152-7
Neuropathologic features of frontotemporal lobar degeneration with ubiquitin-positive inclusions with progranulin gene (PGRN) mutations.
Josephs KA, Ahmed Z, Katsuse O, Parisi JF, Boeve BF, Knopman DS, Petersen RC, Davies P, Duara R, Graff-Radford NR, Uitti RJ, Rademakers R, Adamson J, Baker M, Hutton ML, Dickson DW
Journal of neuropathology and experimental neurology 2007 Feb;66(2):142-51
Journal of neuropathology and experimental neurology 2007 Feb;66(2):142-51
Evidence that TDP-43 is not the major ubiquitinated target within the pathological inclusions of amyotrophic lateral sclerosis.
Sanelli T, Xiao S, Horne P, Bilbao J, Zinman L, Robertson J
Journal of neuropathology and experimental neurology 2007 Dec;66(12):1147-53
Journal of neuropathology and experimental neurology 2007 Dec;66(12):1147-53
Concurrence of TDP-43, tau and alpha-synuclein pathology in brains of Alzheimer's disease and dementia with Lewy bodies.
Higashi S, Iseki E, Yamamoto R, Minegishi M, Hino H, Fujisawa K, Togo T, Katsuse O, Uchikado H, Furukawa Y, Kosaka K, Arai H
Brain research 2007 Dec 12;1184:284-94
Brain research 2007 Dec 12;1184:284-94
Ubiquitinated TDP-43 in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Neumann M, Sampathu DM, Kwong LK, Truax AC, Micsenyi MC, Chou TT, Bruce J, Schuck T, Grossman M, Clark CM, McCluskey LF, Miller BL, Masliah E, Mackenzie IR, Feldman H, Feiden W, Kretzschmar HA, Trojanowski JQ, Lee VM
Science (New York, N.Y.) 2006 Oct 6;314(5796):130-3
Science (New York, N.Y.) 2006 Oct 6;314(5796):130-3
TDP-43 is a component of ubiquitin-positive tau-negative inclusions in frontotemporal lobar degeneration and amyotrophic lateral sclerosis.
Arai T, Hasegawa M, Akiyama H, Ikeda K, Nonaka T, Mori H, Mann D, Tsuchiya K, Yoshida M, Hashizume Y, Oda T
Biochemical and biophysical research communications 2006 Dec 22;351(3):602-11
Biochemical and biophysical research communications 2006 Dec 22;351(3):602-11
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Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- K-562 cells were subjected to SDS PAGE followed by western blot with 10782-2-AP(TARDBP antibody) at dilution of 1:2000
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunofluorescent analysis of HepG2 cells, using TARDBP antibody 10782-2-AP at 1:50 dilution and Rhodamine-labeled goat anti-rabbit IgG (red).
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- IP result of anti-TDP43(10782-2-AP for IP and 60019-2-Ig for Detection).
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human gliomas using 10782-2-AP(TARDBP antibody) at dilution of 1:200 (under 10x lens)
- Sample type
- tissue
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human gliomas using 10782-2-AP(TARDBP antibody) at dilution of 1:200 (under 40x lens)
- Sample type
- tissue
