Antibody data
- Antibody Data
- Antigen structure
- References [130]
- Comments [0]
- Validations
- Western blot [1]
- Immunocytochemistry [1]
- Immunoprecipitation [1]
- Immunohistochemistry [2]
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Validation data
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- Product number
- 12892-1-AP - Provider product page
- Provider
- Proteintech Group
- Proper citation
- Proteintech Cat#12892-1-AP, RRID:AB_2200505
- Product name
- TDP-43 (C-terminal) antibody
- Antibody type
- Polyclonal
- Description
- KD/KO validated TDP-43 (C-terminal) antibody (Cat. #12892-1-AP) is a rabbit polyclonal antibody that shows reactivity with human, mouse, rat and has been validated for the following applications: IF, IHC, IP, WB,ELISA.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Conjugate
- Unconjugated
- Isotype
- IgG
- Vial size
- 20ul, 150ul
Submitted references TDP-43 and FUS mislocalization in VCP mutant motor neurons is reversed by pharmacological inhibition of the VCP D2 ATPase domain.
Parkin Levels Decrease in Fibroblasts With Progranulin (PGRN) Pathogenic Variants and in a Cellular Model of PGRN Deficiency.
TDP-43 mediates SREBF2-regulated gene expression required for oligodendrocyte myelination.
An immune response characterizes early Alzheimer's disease pathology and subjective cognitive impairment in hydrocephalus biopsies.
Age-related demethylation of the TDP-43 autoregulatory region in the human motor cortex.
Interaction of tau with HNRNPA2B1 and N6-methyladenosine RNA mediates the progression of tauopathy.
TDP-43 is essential for Eph receptor-class-specific spinal motor axon trajectory into the limb.
m6A Modified Short RNA Fragments Inhibit Cytoplasmic TLS/FUS Aggregation Induced by Hyperosmotic Stress.
Elevated GFAP isoform expression promotes protein aggregation and compromises astrocyte function.
High-fidelity reconstitution of stress granules and nucleoli in mammalian cellular lysate.
O-GlcNAcylation of TDP-43 suppresses proteinopathies and promotes TDP-43's mRNA splicing activity.
Detection and quantification of novel C-terminal TDP-43 fragments in ALS-TDP.
Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis.
The optineurin/TIA1 pathway inhibits aberrant stress granule formation and reduces ubiquitinated TDP-43.
Hypusination of Eif5a regulates cytoplasmic TDP-43 aggregation and accumulation in a stress-induced cellular model.
TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS.
HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells.
Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization.
Nuclear depletion of RNA-binding protein ELAVL3 (HuC) in sporadic and familial amyotrophic lateral sclerosis.
Decoding distinctive features of plasma extracellular vesicles in amyotrophic lateral sclerosis.
DCTN1 Binds to TDP-43 and Regulates TDP-43 Aggregation.
ATXN1 repeat expansions confer risk for amyotrophic lateral sclerosis and contribute to TDP-43 mislocalization.
Isolation and characterization of antibody fragments selective for human FTD brain derived TDP-43 variants.
Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice.
Stress-Specific Spatiotemporal Responses of RNA-Binding Proteins in Human Stem-Cell-Derived Motor Neurons.
Monoclonal full-length antibody against TAR DNA binding protein 43 reduces related proteinopathy in neurons.
TDP-43 interacts with amyloid-β, inhibits fibrillization, and worsens pathology in a model of Alzheimer's disease.
Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia.
ALS-FTLD-linked mutations of SQSTM1/p62 disrupt selective autophagy and NFE2L2/NRF2 anti-oxidative stress pathway.
TDP-43 promotes the formation of neuromuscular synapses through the regulation of Disc-large expression in Drosophila skeletal muscles.
Integrator restrains paraspeckles assembly by promoting isoform switching of the lncRNA NEAT1.
Coupling of terminal differentiation deficit with neurodegenerative pathology in Vps35-deficient pyramidal neurons.
TDP-43 prevents retrotransposon activation in the Drosophila motor system through regulation of Dicer-2 activity.
Functional characterization of a FUS mutant zebrafish line as a novel genetic model for ALS.
Congenic expression of poly-GA but not poly-PR in mice triggers selective neuron loss and interferon responses found in C9orf72 ALS.
BraInMap Elucidates the Macromolecular Connectivity Landscape of Mammalian Brain.
Cell-to-cell transmission of C9orf72 poly-(Gly-Ala) triggers key features of ALS/FTD.
Non-genetically modified models exhibit TARDBP mRNA increase due to perturbed TDP-43 autoregulation.
C9-ALS/FTD-linked proline-arginine dipeptide repeat protein associates with paraspeckle components and increases paraspeckle formation.
Splicing repression is a major function of TDP-43 in motor neurons.
Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.
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.
Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology.
C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.
Phosphorylated and aggregated TDP-43 with seeding properties are induced upon mutant Huntingtin (mHtt) polyglutamine expression in human cellular models.
Increased Lysosomal Exocytosis Induced by Lysosomal Ca2+ Channel Agonists Protects Human Dopaminergic Neurons from α-Synuclein Toxicity.
Development of an automated capillary nano-immunoassay-Simple Western assay-to quantify total TDP43 protein in human platelet samples.
Heavy Metal Neurotoxicants Induce ALS-Linked TDP-43 Pathology.
TDP-43 proteinopathy in Theiler's murine encephalomyelitis virus infection.
Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity.
TDP-43 Regulates Coupled Dendritic mRNA Transport-Translation Processes in Co-operation with FMRP and Staufen1.
TDP-43-Mediated Toxicity in HEK293T Cells: A Fast and Reproducible Protocol To Be Employed in the Search of New Therapeutic Options against Amyotrophic Lateral Sclerosis.
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.
Differential toxicity of TAR DNA-binding protein 43 isoforms depends on their submitochondrial localization in neuronal cells.
Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.
The proline-arginine repeat protein linked to C9-ALS/FTD causes neuronal toxicity by inhibiting the DEAD-box RNA helicase-mediated ribosome biogenesis.
The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?
The N Termini of TAR DNA-Binding Protein 43 (TDP43) C-Terminal Fragments Influence Degradation, Aggregation Propensity, and Morphology.
TDP-43 and RNA form amyloid-like myo-granules in regenerating muscle.
Microtubules as platforms for probing liquid-liquid phase separation in cells - application to RNA-binding proteins.
Diallyl Trisulfide Protects Motor Neurons from the Neurotoxic Protein TDP-43 via Activating Lysosomal Degradation and the Antioxidant Response.
Identification of aberrantly expressed F-box proteins in squamous-cell lung carcinoma.
Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells.
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.
Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients.
TDP-43 upregulation mediated by the NLRP3 inflammasome induces cognitive impairment in 2 2',4,4'-tetrabromodiphenyl ether (BDE-47)-treated mice.
Dioxins and related environmental contaminants increase TDP-43 levels.
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.
Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis.
Extracellular TDP-43 aggregates target MAPK/MAK/MRK overlapping kinase (MOK) and trigger caspase-3/IL-18 signaling in microglia.
Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.
Tdp-43 cryptic exons are highly variable between cell types.
Immunolocalization of TAR DNA-binding protein of 43 kDa (TDP-43) in mouse seminiferous epithelium.
Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.
TIA1 Mutations in Amyotrophic Lateral Sclerosis and Frontotemporal Dementia Promote Phase Separation and Alter Stress Granule Dynamics.
Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.
Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.
TDP-43 regulates endogenous retrovirus-K viral protein accumulation.
The Lysosomal Trafficking Transmembrane Protein 106B Is Linked to Cell Death.
The Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.
Depletion of TDP-43 decreases fibril and plaque β-amyloid and exacerbates neurodegeneration in an Alzheimer's mouse model.
Cytoplasmic mislocalization of RNA splicing factors and aberrant neuronal gene splicing in TDP-43 transgenic pig brain.
PABPN1 suppresses TDP-43 toxicity in ALS disease models.
Autophagy-linked FYVE protein (Alfy) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS).
Nuclear TDP-43 causes neuronal toxicity by escaping from the inhibitory regulation by hnRNPs.
Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients.
Restoration of progranulin expression rescues cortical neuron generation in an induced pluripotent stem cell model of frontotemporal dementia.
Overexpression of nuclear FUS induces neuronal cell death.
TDP-43 as a possible biomarker for frontotemporal lobar degeneration: a systematic review of existing antibodies.
A nonsense mutation in mouse Tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.
Upregulation of miR-181 decreases c-Fos and SIRT-1 in the hippocampus of 3xTg-AD mice.
Dual vulnerability of TDP-43 to calpain and caspase-3 proteolysis after neurotoxic conditions and traumatic brain injury.
Astrocytic TDP-43 pathology in Alexander disease.
ER-mitochondria associations are regulated by the VAPB-PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43.
Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice.
Metabolism and mis-metabolism of the neuropathological signature protein TDP-43.
Neuroprotection by monocarbonyl dimethoxycurcumin C: ameliorating the toxicity of mutant TDP-43 via HO-1.
A new cellular model of pathological TDP-43: The neurotoxicity of stably expressed CTF25 of TDP-43 depends on the proteasome.
TDP-1, the Caenorhabditis elegans ortholog of TDP-43, limits the accumulation of double-stranded RNA.
Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits.
Heat-shock protein dysregulation is associated with functional and pathological TDP-43 aggregation.
Mice deficient in Epg5 exhibit selective neuronal vulnerability to degeneration.
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.
Tardbpl splicing rescues motor neuron and axonal development in a mutant tardbp zebrafish.
The JNK/c-Jun signaling axis contributes to the TDP-43-induced cell death.
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
The dual functions of the extreme N-terminus of TDP-43 in regulating its biological activity and inclusion formation.
Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology.
Characterization of a series of 4-aminoquinolines that stimulate caspase-7 mediated cleavage of TDP-43 and inhibit its function.
Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line.
TDP-43 toxicity is mediated by the unfolded protein response-unrelated induction of C/EBP homologous protein expression.
Non-human primate model of amyotrophic lateral sclerosis with cytoplasmic mislocalization of TDP-43.
Rodent models of TDP-43: recent advances.
Contrasting pathology of the stress granule proteins TIA-1 and G3BP in tauopathies.
Overexpression of TDP-43 causes partially p53-dependent G2/M arrest and p53-independent cell death in HeLa cells.
Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease.
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
Amyotrophic lateral sclerosis-linked mutant VAPB enhances TDP-43-induced motor neuronal toxicity.
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.
TDP-43-induced death is associated with altered regulation of BIM and Bcl-xL and attenuated by caspase-mediated TDP-43 cleavage.
Mutant TAR DNA-binding protein-43 induces oxidative injury in motor neuron-like cell.
Amyotrophic lateral sclerosis-associated proteins TDP-43 and FUS/TLS function in a common biochemical complex to co-regulate HDAC6 mRNA.
Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin.
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
Wild-type human TDP-43 expression causes TDP-43 phosphorylation, mitochondrial aggregation, motor deficits, and early mortality in transgenic mice.
Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability.
Defective autophagy in neurons and astrocytes from mice deficient in PI(3,5)P2.
Harley J, Hagemann C, Serio A, Patani R
Brain communications 2021;3(3):fcab166
Brain communications 2021;3(3):fcab166
Parkin Levels Decrease in Fibroblasts With Progranulin (PGRN) Pathogenic Variants and in a Cellular Model of PGRN Deficiency.
Gaweda-Walerych K, Walerych D, Berdyński M, Buratti E, Zekanowski C
Frontiers in molecular neuroscience 2021;14:676478
Frontiers in molecular neuroscience 2021;14:676478
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)
An immune response characterizes early Alzheimer's disease pathology and subjective cognitive impairment in hydrocephalus biopsies.
Huang W, Bartosch AM, Xiao H, Maji S, Youth EHH, Flowers X, Leskinen S, Tomljanovic Z, Iodice G, Boyett D, Spinazzi E, Menon V, McGovern RA, McKhann GM, Teich AF
Nature communications 2021 Sep 27;12(1):5659
Nature communications 2021 Sep 27;12(1):5659
Age-related demethylation of the TDP-43 autoregulatory region in the human motor cortex.
Koike Y, Sugai A, Hara N, Ito J, Yokoseki A, Ishihara T, Yamagishi T, Tsuboguchi S, Tada M, Ikeuchi T, Kakita A, Onodera O
Communications biology 2021 Sep 21;4(1):1107
Communications biology 2021 Sep 21;4(1):1107
Interaction of tau with HNRNPA2B1 and N6-methyladenosine RNA mediates the progression of tauopathy.
Jiang L, Lin W, Zhang C, Ash PEA, Verma M, Kwan J, van Vliet E, Yang Z, Cruz AL, Boudeau S, Maziuk BF, Lei S, Song J, Alvarez VE, Hovde S, Abisambra JF, Kuo MH, Kanaan N, Murray ME, Crary JF, Zhao J, Cheng JX, Petrucelli L, Li H, Emili A, Wolozin B
Molecular cell 2021 Oct 21;81(20):4209-4227.e12
Molecular cell 2021 Oct 21;81(20):4209-4227.e12
TDP-43 is essential for Eph receptor-class-specific spinal motor axon trajectory into the limb.
Kao TJ, Wang CY, Hsu TI, Wu YH, Chuang JY, Huang CC, Hsieh CT
Neuroscience research 2021 Oct 20;
Neuroscience research 2021 Oct 20;
m6A Modified Short RNA Fragments Inhibit Cytoplasmic TLS/FUS Aggregation Induced by Hyperosmotic Stress.
Yoneda R, Ueda N, Kurokawa R
International journal of molecular sciences 2021 Oct 12;22(20)
International journal of molecular sciences 2021 Oct 12;22(20)
Elevated GFAP isoform expression promotes protein aggregation and compromises astrocyte function.
Lin NH, Yang AW, Chang CH, Perng MD
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2021 May;35(5):e21614
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2021 May;35(5):e21614
High-fidelity reconstitution of stress granules and nucleoli in mammalian cellular lysate.
Freibaum BD, Messing J, Yang P, Kim HJ, Taylor JP
The Journal of cell biology 2021 Mar 1;220(3)
The Journal of cell biology 2021 Mar 1;220(3)
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
Detection and quantification of novel C-terminal TDP-43 fragments in ALS-TDP.
Feneberg E, Charles PD, Finelli MJ, Scott C, Kessler BM, Fischer R, Ansorge O, Gray E, Talbot K, Turner MR
Brain pathology (Zurich, Switzerland) 2021 Jul;31(4):e12923
Brain pathology (Zurich, Switzerland) 2021 Jul;31(4):e12923
Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis.
Mohassel P, Donkervoort S, Lone MA, Nalls M, Gable K, Gupta SD, Foley AR, Hu Y, Saute JAM, Moreira AL, Kok F, Introna A, Logroscino G, Grunseich C, Nickolls AR, Pourshafie N, Neuhaus SB, Saade D, Gangfuß A, Kölbel H, Piccus Z, Le Pichon CE, Fiorillo C, Ly CV, Töpf A, Brady L, Specht S, Zidell A, Pedro H, Mittelmann E, Thomas FP, Chao KR, Konersman CG, Cho MT, Brandt T, Straub V, Connolly AM, Schara U, Roos A, Tarnopolsky M, Höke A, Brown RH, Lee CH, Hornemann T, Dunn TM, Bönnemann CG
Nature medicine 2021 Jul;27(7):1197-1204
Nature medicine 2021 Jul;27(7):1197-1204
The optineurin/TIA1 pathway inhibits aberrant stress granule formation and reduces ubiquitinated TDP-43.
Kakihana T, Takahashi M, Katsuragi Y, Yamashita SI, Sango J, Kanki T, Onodera O, Fujii M
iScience 2021 Jul 23;24(7):102733
iScience 2021 Jul 23;24(7):102733
Hypusination of Eif5a regulates cytoplasmic TDP-43 aggregation and accumulation in a stress-induced cellular model.
Smeltzer S, Quadri Z, Miller A, Zamudio F, Hunter J, Stewart NJF, Saji S, Lee DC, Chaput D, Selenica MB
Biochimica et biophysica acta. Molecular basis of disease 2021 Jan 1;1867(1):165939
Biochimica et biophysica acta. Molecular basis of disease 2021 Jan 1;1867(1):165939
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
HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells.
Yu H, Lu S, Gasior K, Singh D, Vazquez-Sanchez S, Tapia O, Toprani D, Beccari MS, Yates JR 3rd, Da Cruz S, Newby JM, Lafarga M, Gladfelter AS, Villa E, Cleveland DW
Science (New York, N.Y.) 2021 Feb 5;371(6529)
Science (New York, N.Y.) 2021 Feb 5;371(6529)
Nucleolar stress in C9orf72 and sporadic ALS spinal motor neurons precedes TDP-43 mislocalization.
Aladesuyi Arogundade O, Nguyen S, Leung R, Wainio D, Rodriguez M, Ravits J
Acta neuropathologica communications 2021 Feb 15;9(1):26
Acta neuropathologica communications 2021 Feb 15;9(1):26
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
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
DCTN1 Binds to TDP-43 and Regulates TDP-43 Aggregation.
Deshimaru M, Kinoshita-Kawada M, Kubota K, Watanabe T, Tanaka Y, Hirano S, Ishidate F, Hiramoto M, Ishikawa M, Uehara Y, Okano H, Hirose S, Fujioka S, Iwasaki K, Yuasa-Kawada J, Mishima T, Tsuboi Y
International journal of molecular sciences 2021 Apr 13;22(8)
International journal of molecular sciences 2021 Apr 13;22(8)
ATXN1 repeat expansions confer risk for amyotrophic lateral sclerosis and contribute to TDP-43 mislocalization.
Tazelaar GHP, Boeynaems S, De Decker M, van Vugt JJFA, Kool L, Goedee HS, McLaughlin RL, Sproviero W, Iacoangeli A, Moisse M, Jacquemyn M, Daelemans D, Dekker AM, van der Spek RA, Westeneng HJ, Kenna KP, Assialioui A, Da Silva N, Project MinE ALS Sequencing Consortium ., Povedano M, Pardina JSM, Hardiman O, Salachas F, Millecamps S, Vourc'h P, Corcia P, Couratier P, Morrison KE, Shaw PJ, Shaw CE, Pasterkamp RJ, Landers JE, Van Den Bosch L, Robberecht W, Al-Chalabi A, van den Berg LH, Van Damme P, Veldink JH, van Es MA
Brain communications 2020;2(2):fcaa064
Brain communications 2020;2(2):fcaa064
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
Loss of TMEM106B potentiates lysosomal and FTLD-like pathology in progranulin-deficient mice.
Werner G, Damme M, Schludi M, Gnörich J, Wind K, Fellerer K, Wefers B, Wurst W, Edbauer D, Brendel M, Haass C, Capell A
EMBO reports 2020 Oct 5;21(10):e50241
EMBO reports 2020 Oct 5;21(10):e50241
Stress-Specific Spatiotemporal Responses of RNA-Binding Proteins in Human Stem-Cell-Derived Motor Neurons.
Harley J, Patani R
International journal of molecular sciences 2020 Nov 6;21(21)
International journal of molecular sciences 2020 Nov 6;21(21)
Monoclonal full-length antibody against TAR DNA binding protein 43 reduces related proteinopathy in neurons.
Pozzi S, Codron P, Soucy G, Renaud L, Cordeau PJ, Dutta K, Bareil C, Julien JP
JCI insight 2020 Nov 5;5(21)
JCI insight 2020 Nov 5;5(21)
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
Truncated stathmin-2 is a marker of TDP-43 pathology in frontotemporal dementia.
Prudencio M, Humphrey J, Pickles S, Brown AL, Hill SE, Kachergus JM, Shi J, Heckman MG, Spiegel MR, Cook C, Song Y, Yue M, Daughrity LM, Carlomagno Y, Jansen-West K, de Castro CF, DeTure M, Koga S, Wang YC, Sivakumar P, Bodo C, Candalija A, Talbot K, Selvaraj BT, Burr K, Chandran S, Newcombe J, Lashley T, Hubbard I, Catalano D, Kim D, Propp N, Fennessey S, NYGC ALS Consortium., Fagegaltier D, Phatnani H, Secrier M, Fisher EM, Oskarsson B, van Blitterswijk M, Rademakers R, Graff-Radford NR, Boeve BF, Knopman DS, Petersen RC, Josephs KA, Thompson EA, Raj T, Ward M, Dickson DW, Gendron TF, Fratta P, Petrucelli L
The Journal of clinical investigation 2020 Nov 2;130(11):6080-6092
The Journal of clinical investigation 2020 Nov 2;130(11):6080-6092
ALS-FTLD-linked mutations of SQSTM1/p62 disrupt selective autophagy and NFE2L2/NRF2 anti-oxidative stress pathway.
Deng Z, Lim J, Wang Q, Purtell K, Wu S, Palomo GM, Tan H, Manfredi G, Zhao Y, Peng J, Hu B, Chen S, Yue Z
Autophagy 2020 May;16(5):917-931
Autophagy 2020 May;16(5):917-931
TDP-43 promotes the formation of neuromuscular synapses through the regulation of Disc-large expression in Drosophila skeletal muscles.
Strah N, Romano G, Introna C, Klima R, Marzullo M, Ciapponi L, Megighian A, Nizzardo M, Feiguin F
BMC biology 2020 Mar 26;18(1):34
BMC biology 2020 Mar 26;18(1):34
Integrator restrains paraspeckles assembly by promoting isoform switching of the lncRNA NEAT1.
Barra J, Gaidosh GS, Blumenthal E, Beckedorff F, Tayari MM, Kirstein N, Karakach TK, Jensen TH, Impens F, Gevaert K, Leucci E, Shiekhattar R, Marine JC
Science advances 2020 Jul;6(27):eaaz9072
Science advances 2020 Jul;6(27):eaaz9072
Coupling of terminal differentiation deficit with neurodegenerative pathology in Vps35-deficient pyramidal neurons.
Tang FL, Zhao L, Zhao Y, Sun D, Zhu XJ, Mei L, Xiong WC
Cell death and differentiation 2020 Jul;27(7):2099-2116
Cell death and differentiation 2020 Jul;27(7):2099-2116
TDP-43 prevents retrotransposon activation in the Drosophila motor system through regulation of Dicer-2 activity.
Romano G, Klima R, Feiguin F
BMC biology 2020 Jul 3;18(1):82
BMC biology 2020 Jul 3;18(1):82
Functional characterization of a FUS mutant zebrafish line as a novel genetic model for ALS.
Bourefis AR, Campanari ML, Buee-Scherrer V, Kabashi E
Neurobiology of disease 2020 Aug;142:104935
Neurobiology of disease 2020 Aug;142:104935
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
BraInMap Elucidates the Macromolecular Connectivity Landscape of Mammalian Brain.
Pourhaghighi R, Ash PEA, Phanse S, Goebels F, Hu LZM, Chen S, Zhang Y, Wierbowski SD, Boudeau S, Moutaoufik MT, Malty RH, Malolepsza E, Tsafou K, Nathan A, Cromar G, Guo H, Abdullatif AA, Apicco DJ, Becker LA, Gitler AD, Pulst SM, Youssef A, Hekman R, Havugimana PC, White CA, Blum BC, Ratti A, Bryant CD, Parkinson J, Lage K, Babu M, Yu H, Bader GD, Wolozin B, Emili A
Cell systems 2020 Apr 22;10(4):333-350.e14
Cell systems 2020 Apr 22;10(4):333-350.e14
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
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
C9-ALS/FTD-linked proline-arginine dipeptide repeat protein associates with paraspeckle components and increases paraspeckle formation.
Suzuki H, Shibagaki Y, Hattori S, Matsuoka M
Cell death & disease 2019 Oct 3;10(10):746
Cell death & disease 2019 Oct 3;10(10):746
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
Transcription elongation factor AFF2/FMR2 regulates expression of expanded GGGGCC repeat-containing C9ORF72 allele in ALS/FTD.
Yuva-Aydemir Y, Almeida S, Krishnan G, Gendron TF, Gao FB
Nature communications 2019 Nov 29;10(1):5466
Nature communications 2019 Nov 29;10(1):5466
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
Chronic optogenetic induction of stress granules is cytotoxic and reveals the evolution of ALS-FTD pathology.
Zhang P, Fan B, Yang P, Temirov J, Messing J, Kim HJ, Taylor JP
eLife 2019 Mar 20;8
eLife 2019 Mar 20;8
C9ORF72-ALS/FTD-associated poly(GR) binds Atp5a1 and compromises mitochondrial function in vivo.
Choi SY, Lopez-Gonzalez R, Krishnan G, Phillips HL, Li AN, Seeley WW, Yao WD, Almeida S, Gao FB
Nature neuroscience 2019 Jun;22(6):851-862
Nature neuroscience 2019 Jun;22(6):851-862
Phosphorylated and aggregated TDP-43 with seeding properties are induced upon mutant Huntingtin (mHtt) polyglutamine expression in human cellular models.
Coudert L, Nonaka T, Bernard E, Hasegawa M, Schaeffer L, Leblanc P
Cellular and molecular life sciences : CMLS 2019 Jul;76(13):2615-2632
Cellular and molecular life sciences : CMLS 2019 Jul;76(13):2615-2632
Increased Lysosomal Exocytosis Induced by Lysosomal Ca2+ Channel Agonists Protects Human Dopaminergic Neurons from α-Synuclein Toxicity.
Tsunemi T, Perez-Rosello T, Ishiguro Y, Yoroisaka A, Jeon S, Hamada K, Rammonhan M, Wong YC, Xie Z, Akamatsu W, Mazzulli JR, Surmeier DJ, Hattori N, Krainc D
The Journal of neuroscience : the official journal of the Society for Neuroscience 2019 Jul 17;39(29):5760-5772
The Journal of neuroscience : the official journal of the Society for Neuroscience 2019 Jul 17;39(29):5760-5772
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
Heavy Metal Neurotoxicants Induce ALS-Linked TDP-43 Pathology.
Ash PEA, Dhawan U, Boudeau S, Lei S, Carlomagno Y, Knobel M, Al Mohanna LFA, Boomhower SR, Newland MC, Sherr DH, Wolozin B
Toxicological sciences : an official journal of the Society of Toxicology 2019 Jan 1;167(1):105-115
Toxicological sciences : an official journal of the Society of Toxicology 2019 Jan 1;167(1):105-115
TDP-43 proteinopathy in Theiler's murine encephalomyelitis virus infection.
Masaki K, Sonobe Y, Ghadge G, Pytel P, Roos RP
PLoS pathogens 2019 Feb;15(2):e1007574
PLoS pathogens 2019 Feb;15(2):e1007574
Heterochromatin anomalies and double-stranded RNA accumulation underlie C9orf72 poly(PR) toxicity.
Zhang YJ, Guo L, Gonzales PK, Gendron TF, Wu Y, Jansen-West K, O'Raw AD, Pickles SR, Prudencio M, Carlomagno Y, Gachechiladze MA, Ludwig C, Tian R, Chew J, DeTure M, Lin WL, Tong J, Daughrity LM, Yue M, Song Y, Andersen JW, Castanedes-Casey M, Kurti A, Datta A, Antognetti G, McCampbell A, Rademakers R, Oskarsson B, Dickson DW, Kampmann M, Ward ME, Fryer JD, Link CD, Shorter J, Petrucelli L
Science (New York, N.Y.) 2019 Feb 15;363(6428)
Science (New York, N.Y.) 2019 Feb 15;363(6428)
TDP-43 Regulates Coupled Dendritic mRNA Transport-Translation Processes in Co-operation with FMRP and Staufen1.
Chu JF, Majumder P, Chatterjee B, Huang SL, Shen CJ
Cell reports 2019 Dec 3;29(10):3118-3133.e6
Cell reports 2019 Dec 3;29(10):3118-3133.e6
TDP-43-Mediated Toxicity in HEK293T Cells: A Fast and Reproducible Protocol To Be Employed in the Search of New Therapeutic Options against Amyotrophic Lateral Sclerosis.
Lanznaster D, Bourgeais J, Bruno C, Hergesheimer RC, Thepault RA, Vourc'h P, Corcia P, Andres CR, Herault O, Blasco H
Cells 2019 Dec 26;9(1)
Cells 2019 Dec 26;9(1)
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
Differential toxicity of TAR DNA-binding protein 43 isoforms depends on their submitochondrial localization in neuronal cells.
Salvatori I, Ferri A, Scaricamazza S, Giovannelli I, Serrano A, Rossi S, D'Ambrosi N, Cozzolino M, Giulio AD, Moreno S, Valle C, Carrì MT
Journal of neurochemistry 2018 Sep;146(5):585-597
Journal of neurochemistry 2018 Sep;146(5):585-597
Senataxin mutations elicit motor neuron degeneration phenotypes and yield TDP-43 mislocalization in ALS4 mice and human patients.
Bennett CL, Dastidar SG, Ling SC, Malik B, Ashe T, Wadhwa M, Miller DB, Lee C, Mitchell MB, van Es MA, Grunseich C, Chen Y, Sopher BL, Greensmith L, Cleveland DW, La Spada AR
Acta neuropathologica 2018 Sep;136(3):425-443
Acta neuropathologica 2018 Sep;136(3):425-443
The proline-arginine repeat protein linked to C9-ALS/FTD causes neuronal toxicity by inhibiting the DEAD-box RNA helicase-mediated ribosome biogenesis.
Suzuki H, Shibagaki Y, Hattori S, Matsuoka M
Cell death & disease 2018 Sep 24;9(10):975
Cell death & disease 2018 Sep 24;9(10):975
The heterozygous R155C VCP mutation: Toxic in humans! Harmless in mice?
Clemen CS, Winter L, Strucksberg KH, Berwanger C, Türk M, Kornblum C, Florin A, Aguilar-Pimentel JA, Amarie OV, Becker L, Garrett L, Hans W, Moreth K, Neff F, Pingen L, Rathkolb B, Rácz I, Rozman J, Treise I, Fuchs H, Gailus-Durner V, de Angelis MH, Vorgerd M, Eichinger L, Schröder R
Biochemical and biophysical research communications 2018 Sep 18;503(4):2770-2777
Biochemical and biophysical research communications 2018 Sep 18;503(4):2770-2777
The N Termini of TAR DNA-Binding Protein 43 (TDP43) C-Terminal Fragments Influence Degradation, Aggregation Propensity, and Morphology.
Kasu YAT, Alemu S, Lamari A, Loew N, Brower CS
Molecular and cellular biology 2018 Oct 1;38(19)
Molecular and cellular biology 2018 Oct 1;38(19)
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
Microtubules as platforms for probing liquid-liquid phase separation in cells - application to RNA-binding proteins.
Maucuer A, Desforges B, Joshi V, Boca M, Kretov DA, Hamon L, Bouhss A, Curmi PA, Pastré D
Journal of cell science 2018 Jun 11;131(11)
Journal of cell science 2018 Jun 11;131(11)
Diallyl Trisulfide Protects Motor Neurons from the Neurotoxic Protein TDP-43 via Activating Lysosomal Degradation and the Antioxidant Response.
Liu C, Leng B, Li Y, Jiang H, Duan W, Guo Y, Li C, Hong K
Neurochemical research 2018 Dec;43(12):2304-2312
Neurochemical research 2018 Dec;43(12):2304-2312
Identification of aberrantly expressed F-box proteins in squamous-cell lung carcinoma.
Wang K, Qu X, Liu S, Yang X, Bie F, Wang Y, Huang C, Du J
Journal of cancer research and clinical oncology 2018 Aug;144(8):1509-1521
Journal of cancer research and clinical oncology 2018 Aug;144(8):1509-1521
Tdp-25 Routing to Autophagy and Proteasome Ameliorates its Aggregation in Amyotrophic Lateral Sclerosis Target Cells.
Cicardi ME, Cristofani R, Rusmini P, Meroni M, Ferrari V, Vezzoli G, Tedesco B, Piccolella M, Messi E, Galbiati M, Boncoraglio A, Carra S, Crippa V, Poletti A
Scientific reports 2018 Aug 17;8(1):12390
Scientific reports 2018 Aug 17;8(1):12390
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
Repetitive element transcripts are elevated in the brain of C9orf72 ALS/FTLD patients.
Prudencio M, Gonzales PK, Cook CN, Gendron TF, Daughrity LM, Song Y, Ebbert MTW, van Blitterswijk M, Zhang YJ, Jansen-West K, Baker MC, DeTure M, Rademakers R, Boylan KB, Dickson DW, Petrucelli L, Link CD
Human molecular genetics 2017 Sep 1;26(17):3421-3431
Human molecular genetics 2017 Sep 1;26(17):3421-3431
TDP-43 upregulation mediated by the NLRP3 inflammasome induces cognitive impairment in 2 2',4,4'-tetrabromodiphenyl ether (BDE-47)-treated mice.
Zhuang J, Wen X, Zhang YQ, Shan Q, Zhang ZF, Zheng GH, Fan SH, Li MQ, Wu DM, Hu B, Lu J, Zheng YL
Brain, behavior, and immunity 2017 Oct;65:99-110
Brain, behavior, and immunity 2017 Oct;65:99-110
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
Phase Separation of C9orf72 Dipeptide Repeats Perturbs Stress Granule Dynamics.
Boeynaems S, Bogaert E, Kovacs D, Konijnenberg A, Timmerman E, Volkov A, Guharoy M, De Decker M, Jaspers T, Ryan VH, Janke AM, Baatsen P, Vercruysse T, Kolaitis RM, Daelemans D, Taylor JP, Kedersha N, Anderson P, Impens F, Sobott F, Schymkowitz J, Rousseau F, Fawzi NL, Robberecht W, Van Damme P, Tompa P, Van Den Bosch L
Molecular cell 2017 Mar 16;65(6):1044-1055.e5
Molecular cell 2017 Mar 16;65(6):1044-1055.e5
Mutant TDP-43 within motor neurons drives disease onset but not progression in amyotrophic lateral sclerosis.
Ditsworth D, Maldonado M, McAlonis-Downes M, Sun S, Seelman A, Drenner K, Arnold E, Ling SC, Pizzo D, Ravits J, Cleveland DW, Da Cruz S
Acta neuropathologica 2017 Jun;133(6):907-922
Acta neuropathologica 2017 Jun;133(6):907-922
Extracellular TDP-43 aggregates target MAPK/MAK/MRK overlapping kinase (MOK) and trigger caspase-3/IL-18 signaling in microglia.
Leal-Lasarte MM, Franco JM, Labrador-Garrido A, Pozo D, Roodveldt C
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2017 Jul;31(7):2797-2816
FASEB journal : official publication of the Federation of American Societies for Experimental Biology 2017 Jul;31(7):2797-2816
Suppression of C9orf72 RNA repeat-induced neurotoxicity by the ALS-associated RNA-binding protein Zfp106.
Celona B, Dollen JV, Vatsavayai SC, Kashima R, Johnson JR, Tang AA, Hata A, Miller BL, Huang EJ, Krogan NJ, Seeley WW, Black BL
eLife 2017 Jan 10;6
eLife 2017 Jan 10;6
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
Immunolocalization of TAR DNA-binding protein of 43 kDa (TDP-43) in mouse seminiferous epithelium.
Osuru HP, Pramoonjago P, Abhyankar MM, Swanson E, Roker LA, Cathro H, Reddi PP
Molecular reproduction and development 2017 Aug;84(8):675-685
Molecular reproduction and development 2017 Aug;84(8):675-685
Spinal poly-GA inclusions in a C9orf72 mouse model trigger motor deficits and inflammation without neuron loss.
Schludi MH, Becker L, Garrett L, Gendron TF, Zhou Q, Schreiber F, Popper B, Dimou L, Strom TM, Winkelmann J, von Thaden A, Rentzsch K, May S, Michaelsen M, Schwenk BM, Tan J, Schoser B, Dieterich M, Petrucelli L, Hölter SM, Wurst W, Fuchs H, Gailus-Durner V, de Angelis MH, Klopstock T, Arzberger T, Edbauer D
Acta neuropathologica 2017 Aug;134(2):241-254
Acta neuropathologica 2017 Aug;134(2):241-254
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
Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice.
Becker LA, Huang B, Bieri G, Ma R, Knowles DA, Jafar-Nejad P, Messing J, Kim HJ, Soriano A, Auburger G, Pulst SM, Taylor JP, Rigo F, Gitler AD
Nature 2017 Apr 20;544(7650):367-371
Nature 2017 Apr 20;544(7650):367-371
Mislocated FUS is sufficient for gain-of-toxic-function amyotrophic lateral sclerosis phenotypes in mice.
Shiihashi G, Ito D, Yagi T, Nihei Y, Ebine T, Suzuki N
Brain : a journal of neurology 2016 Sep;139(Pt 9):2380-94
Brain : a journal of neurology 2016 Sep;139(Pt 9):2380-94
TDP-43 regulates endogenous retrovirus-K viral protein accumulation.
Manghera M, Ferguson-Parry J, Douville RN
Neurobiology of disease 2016 Oct;94:226-36
Neurobiology of disease 2016 Oct;94:226-36
The Lysosomal Trafficking Transmembrane Protein 106B Is Linked to Cell Death.
Suzuki H, Matsuoka M
The Journal of biological chemistry 2016 Oct 7;291(41):21448-21460
The Journal of biological chemistry 2016 Oct 7;291(41):21448-21460
The Myoblast C2C12 Transfected with Mutant Valosin-Containing Protein Exhibits Delayed Stress Granule Resolution on Oxidative Stress.
Rodriguez-Ortiz CJ, Flores JC, Valenzuela JA, Rodriguez GJ, Zumkehr J, Tran DN, Kimonis VE, Kitazawa M
The American journal of pathology 2016 Jun;186(6):1623-34
The American journal of pathology 2016 Jun;186(6):1623-34
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
Cytoplasmic mislocalization of RNA splicing factors and aberrant neuronal gene splicing in TDP-43 transgenic pig brain.
Wang G, Yang H, Yan S, Wang CE, Liu X, Zhao B, Ouyang Z, Yin P, Liu Z, Zhao Y, Liu T, Fan N, Guo L, Li S, Li XJ, Lai L
Molecular neurodegeneration 2015 Sep 3;10:42
Molecular neurodegeneration 2015 Sep 3;10:42
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
Autophagy-linked FYVE protein (Alfy) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS).
Han H, Wei W, Duan W, Guo Y, Li Y, Wang J, Bi Y, Li C
In vitro cellular & developmental biology. Animal 2015 Mar;51(3):249-63
In vitro cellular & developmental biology. Animal 2015 Mar;51(3):249-63
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
Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients.
Paré B, Touzel-Deschênes L, Lamontagne R, Lamarre MS, Scott FD, Khuong HT, Dion PA, Bouchard JP, Gould P, Rouleau GA, Dupré N, Berthod F, Gros-Louis F
Acta neuropathologica communications 2015 Jan 31;3:5
Acta neuropathologica communications 2015 Jan 31;3:5
Restoration of progranulin expression rescues cortical neuron generation in an induced pluripotent stem cell model of frontotemporal dementia.
Raitano S, Ordovàs L, De Muynck L, Guo W, Espuny-Camacho I, Geraerts M, Khurana S, Vanuytsel K, Tóth BI, Voets T, Vandenberghe R, Cathomen T, Van Den Bosch L, Vanderhaeghen P, Van Damme P, Verfaillie CM
Stem cell reports 2015 Jan 13;4(1):16-24
Stem cell reports 2015 Jan 13;4(1):16-24
Overexpression of nuclear FUS induces neuronal cell death.
Suzuki H, Matsuoka M
Neuroscience 2015 Feb 26;287:113-24
Neuroscience 2015 Feb 26;287:113-24
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
A nonsense mutation in mouse Tardbp affects TDP43 alternative splicing activity and causes limb-clasping and body tone defects.
Ricketts T, McGoldrick P, Fratta P, de Oliveira HM, Kent R, Phatak V, Brandner S, Blanco G, Greensmith L, Acevedo-Arozena A, Fisher EM
PloS one 2014;9(1):e85962
PloS one 2014;9(1):e85962
Upregulation of miR-181 decreases c-Fos and SIRT-1 in the hippocampus of 3xTg-AD mice.
Rodriguez-Ortiz CJ, Baglietto-Vargas D, Martinez-Coria H, LaFerla FM, Kitazawa M
Journal of Alzheimer's disease : JAD 2014;42(4):1229-38
Journal of Alzheimer's disease : JAD 2014;42(4):1229-38
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
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
ER-mitochondria associations are regulated by the VAPB-PTPIP51 interaction and are disrupted by ALS/FTD-associated TDP-43.
Stoica R, De Vos KJ, Paillusson S, Mueller S, Sancho RM, Lau KF, Vizcay-Barrena G, Lin WL, Xu YF, Lewis J, Dickson DW, Petrucelli L, Mitchell JC, Shaw CE, Miller CC
Nature communications 2014 Jun 3;5:3996
Nature communications 2014 Jun 3;5:3996
Prevention of intestinal obstruction reveals progressive neurodegeneration in mutant TDP-43 (A315T) mice.
Herdewyn S, Cirillo C, Van Den Bosch L, Robberecht W, Vanden Berghe P, Van Damme P
Molecular neurodegeneration 2014 Jun 17;9:24
Molecular neurodegeneration 2014 Jun 17;9:24
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
Neuroprotection by monocarbonyl dimethoxycurcumin C: ameliorating the toxicity of mutant TDP-43 via HO-1.
Duan W, Guo Y, Xiao J, Chen X, Li Z, Han H, Li C
Molecular neurobiology 2014 Feb;49(1):368-79
Molecular neurobiology 2014 Feb;49(1):368-79
A new cellular model of pathological TDP-43: The neurotoxicity of stably expressed CTF25 of TDP-43 depends on the proteasome.
Liu Y, Duan W, Guo Y, Li Z, Han H, Zhang S, Yuan P, Li C
Neuroscience 2014 Dec 5;281:88-98
Neuroscience 2014 Dec 5;281:88-98
TDP-1, the Caenorhabditis elegans ortholog of TDP-43, limits the accumulation of double-stranded RNA.
Saldi TK, Ash PE, Wilson G, Gonzales P, Garrido-Lecca A, Roberts CM, Dostal V, Gendron TF, Stein LD, Blumenthal T, Petrucelli L, Link CD
The EMBO journal 2014 Dec 17;33(24):2947-66
The EMBO journal 2014 Dec 17;33(24):2947-66
Interaction of transactive response DNA binding protein 43 with nuclear factor κB in mild cognitive impairment with episodic memory deficits.
Ohta Y, Tremblay C, Schneider JA, Bennett DA, Calon F, Julien JP
Acta neuropathologica communications 2014 Apr 1;2:37
Acta neuropathologica communications 2014 Apr 1;2:37
Heat-shock protein dysregulation is associated with functional and pathological TDP-43 aggregation.
Chang HY, Hou SC, Way TD, Wong CH, Wang IF
Nature communications 2013;4:2757
Nature communications 2013;4:2757
Mice deficient in Epg5 exhibit selective neuronal vulnerability to degeneration.
Zhao H, Zhao YG, Wang X, Xu L, Miao L, Feng D, Chen Q, Kovács AL, Fan D, Zhang H
The Journal of cell biology 2013 Mar 18;200(6):731-41
The Journal of cell biology 2013 Mar 18;200(6):731-41
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
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
The JNK/c-Jun signaling axis contributes to the TDP-43-induced cell death.
Suzuki H, Matsuoka M
Molecular and cellular biochemistry 2013 Jan;372(1-2):241-8
Molecular and cellular biochemistry 2013 Jan;372(1-2):241-8
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43.
Arnold ES, Ling SC, Huelga SC, Lagier-Tourenne C, Polymenidou M, Ditsworth D, Kordasiewicz HB, McAlonis-Downes M, Platoshyn O, Parone PA, Da Cruz S, Clutario KM, Swing D, Tessarollo L, Marsala M, Shaw CE, Yeo GW, Cleveland DW
Proceedings of the National Academy of Sciences of the United States of America 2013 Feb 19;110(8):E736-45
Proceedings of the National Academy of Sciences of the United States of America 2013 Feb 19;110(8):E736-45
Neuronal-specific overexpression of a mutant valosin-containing protein associated with IBMPFD promotes aberrant ubiquitin and TDP-43 accumulation and cognitive dysfunction in transgenic mice.
Rodriguez-Ortiz CJ, Hoshino H, Cheng D, Liu-Yescevitz L, Blurton-Jones M, Wolozin B, LaFerla FM, Kitazawa M
The American journal of pathology 2013 Aug;183(2):504-15
The American journal of pathology 2013 Aug;183(2):504-15
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
Mutations in SQSTM1 encoding p62 in amyotrophic lateral sclerosis: genetics and neuropathology.
Teyssou E, Takeda T, Lebon V, Boillée S, Doukouré B, Bataillon G, Sazdovitch V, Cazeneuve C, Meininger V, LeGuern E, Salachas F, Seilhean D, Millecamps S
Acta neuropathologica 2013 Apr;125(4):511-22
Acta neuropathologica 2013 Apr;125(4):511-22
Characterization of a series of 4-aminoquinolines that stimulate caspase-7 mediated cleavage of TDP-43 and inhibit its function.
Cassel JA, McDonnell ME, Velvadapu V, Andrianov V, Reitz AB
Biochimie 2012 Sep;94(9):1974-81
Biochimie 2012 Sep;94(9):1974-81
Full-length TDP-43 and its C-terminal fragments activate mitophagy in NSC34 cell line.
Hong K, Li Y, Duan W, Guo Y, Jiang H, Li W, Li C
Neuroscience letters 2012 Nov 21;530(2):144-9
Neuroscience letters 2012 Nov 21;530(2):144-9
TDP-43 toxicity is mediated by the unfolded protein response-unrelated induction of C/EBP homologous protein expression.
Suzuki H, Matsuoka M
Journal of neuroscience research 2012 Mar;90(3):641-7
Journal of neuroscience research 2012 Mar;90(3):641-7
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
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
Contrasting pathology of the stress granule proteins TIA-1 and G3BP in tauopathies.
Vanderweyde T, Yu H, Varnum M, Liu-Yesucevitz L, Citro A, Ikezu T, Duff K, Wolozin B
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Jun 13;32(24):8270-83
The Journal of neuroscience : the official journal of the Society for Neuroscience 2012 Jun 13;32(24):8270-83
Overexpression of TDP-43 causes partially p53-dependent G2/M arrest and p53-independent cell death in HeLa cells.
Lee K, Suzuki H, Aiso S, Matsuoka M
Neuroscience letters 2012 Jan 11;506(2):271-6
Neuroscience letters 2012 Jan 11;506(2):271-6
Accumulation of transactive response DNA binding protein 43 in mild cognitive impairment and Alzheimer disease.
Tremblay C, St-Amour I, Schneider J, Bennett DA, Calon F
Journal of neuropathology and experimental neurology 2011 Sep;70(9):788-98
Journal of neuropathology and experimental neurology 2011 Sep;70(9):788-98
Expression of mutant TDP-43 induces neuronal dysfunction in transgenic mice.
Xu YF, Zhang YJ, Lin WL, Cao X, Stetler C, Dickson DW, Lewis J, Petrucelli L
Molecular neurodegeneration 2011 Oct 26;6:73
Molecular neurodegeneration 2011 Oct 26;6:73
Amyotrophic lateral sclerosis-linked mutant VAPB enhances TDP-43-induced motor neuronal toxicity.
Suzuki H, Matsuoka M
Journal of neurochemistry 2011 Dec;119(5):1099-107
Journal of neurochemistry 2011 Dec;119(5):1099-107
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
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
Mutant TAR DNA-binding protein-43 induces oxidative injury in motor neuron-like cell.
Duan W, Li X, Shi J, Guo Y, Li Z, Li C
Neuroscience 2010 Sep 15;169(4):1621-9
Neuroscience 2010 Sep 15;169(4):1621-9
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
Sortilin-mediated endocytosis determines levels of the frontotemporal dementia protein, progranulin.
Hu F, Padukkavidana T, Vægter CB, Brady OA, Zheng Y, Mackenzie IR, Feldman HH, Nykjaer A, Strittmatter SM
Neuron 2010 Nov 18;68(4):654-67
Neuron 2010 Nov 18;68(4):654-67
TDP-43, a neuro-pathosignature factor, is essential for early mouse embryogenesis.
Wu LS, Cheng WC, Hou SC, Yan YT, Jiang ST, Shen CK
Genesis (New York, N.Y. : 2000) 2010 Jan;48(1):56-62
Genesis (New York, N.Y. : 2000) 2010 Jan;48(1):56-62
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
Rapamycin rescues TDP-43 mislocalization and the associated low molecular mass neurofilament instability.
Caccamo A, Majumder S, Deng JJ, Bai Y, Thornton FB, Oddo S
The Journal of biological chemistry 2009 Oct 2;284(40):27416-24
The Journal of biological chemistry 2009 Oct 2;284(40):27416-24
Defective autophagy in neurons and astrocytes from mice deficient in PI(3,5)P2.
Ferguson CJ, Lenk GM, Meisler MH
Human molecular genetics 2009 Dec 15;18(24):4868-78
Human molecular genetics 2009 Dec 15;18(24):4868-78
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Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- HeLa cells were subjected to SDS PAGE followed by western blot with 12892-1-AP(TARDBP antibody) at dilution of 1:1200
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunofluorescent analysis of A549 cells, using TARDBP antibody 12892-1-AP at 1:50 dilution and Rhodamine-labeled goat anti-rabbit IgG (red). Blue pseudocolor = DAPI (fluorescent DNA dye).
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- IP Result of anti-TARDBP (IP:12892-1-AP, 3ug; Detection:12892-1-AP 1:1000) with HeLa cells lysate 3000ug.
- Sample type
- cell line
Supportive validation
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human lung cancer using 12892-1-AP(TARDBP antibody) at dilution of 1:100 (under 10x lens)
- Sample type
- tissue
- Submitted by
- Proteintech Group (provider)
- Main image
- Experimental details
- Immunohistochemical of paraffin-embedded human lung cancer using 12892-1-AP(TARDBP antibody) at dilution of 1:100 (under 40x lens)
- Sample type
- tissue