HPA008784

antibody from Atlas Antibodies

Targeting: FUS ALS6, FUS1, hnRNP-P2, HNRNPP2, TLS

Provider product page for HPA008784

Western blot

Immunocytochemistry

Immunohistochemistry

Antibody data

Antibody Data
Antigen structure
References [42]
Comments [0]
Validations
Western blot [1]
Immunohistochemistry [1]

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Product number: HPA008784 - Provider product page
Provider: Atlas Antibodies
Proper citation: Atlas Antibodies Cat#HPA008784, RRID:AB_1849181
Product name: Anti-FUS
Antibody type: Polyclonal
Description: Polyclonal Antibody against Human FUS, Gene description: FUS RNA binding protein, Alternative Gene Names: ALS6, FUS1, hnRNP-P2, HNRNPP2, TLS, Validated applications: ICC, IHC, WB, Uniprot ID: P35637, Storage: Store at +4°C for short term storage. Long time storage is recommended at -20°C.
Reactivity: Human, Mouse, Rat
Host: Rabbit
Conjugate: Unconjugated
Isotype: IgG
Vial size: 100 µl
Concentration: 0.2 mg/ml
Storage: Store at +4°C for short term storage. Long time storage is recommended at -20°C.
Handling: The antibody solution should be gently mixed before use.

FUS protein structure - HPA008784 shown in red.

Submitted references Psychiatric symptoms of frontotemporal dementia and subcortical (co-)pathology burden: new insights

Scarioni M, Gami-Patel P, Peeters C, de Koning F, Seelaar H, Mol M, van Swieten J, Rozemuller A, Hoozemans J, Pijnenburg Y, Dijkstra A
Brain 2023;146(1):307-320

Targeting RACK1 to alleviate TDP-43 and FUS proteinopathy-mediated suppression of protein translation and neurodegeneration

Zhao B, Cowan C, Coutts J, Christy D, Saraph A, Hsueh S, Plotkin S, Mackenzie I, Kaplan J, Cashman N
Acta Neuropathologica Communications 2023;11(1)

Neuronal dysfunction caused by FUSR521G promotes ALS-associated phenotypes that are attenuated by NF-κB inhibition

Pelaez M, Desmeules A, Gelon P, Glasson B, Marcadet L, Rodgers A, Phaneuf D, Pozzi S, Dutchak P, Julien J, Sephton C
Acta Neuropathologica Communications 2023;11(1)

Acid sphingomyelinase inhibition improves motor behavioral deficits and neuronal loss in an amyotrophic lateral sclerosis mouse model

Choi B, Park K, Park M, Huang E, Kim S, Bae J, Jin H
BMB Reports 2022;55(12):621-626

NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility

Gleixner A, Verdone B, Otte C, Anderson E, Ramesh N, Shapiro O, Gale J, Mauna J, Mann J, Copley K, Daley E, Ortega J, Cicardi M, Kiskinis E, Kofler J, Pandey U, Trotti D, Donnelly C
Nature Communications 2022;13(1)

Proteomic analysis of heat-stable proteins revealed an increased proportion of proteins with compositionally biased regions

Park H, Yamanaka T, Nukina N
Scientific Reports 2022;12(1)

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 M, Feuillette S, Audebrand A, Lecourtois M, Rousseau S, Richard A, Cassinari K, Deramecourt V, Duyckaerts C, Boland A, Deleuze J, Meyer V, Clarimon Echavarria J, Gelpi E, Akiyama H, Hasegawa M, Kawakami I, Wong T, Van Rooij J, Van Swieten J, Campion D, Dutchak P, Wallon D, Lavoie-Cardinal F, Laquerrière A, Rovelet-Lecrux A, Sephton C
Acta Neuropathologica Communications 2022;10(1)

A novel temporal‐predominant neuro‐astroglial tauopathy associated with TMEM106B gene polymorphism in FTLD/ALS‐TDP

Llibre‐Guerra J, Lee S, Suemoto C, Ehrenberg A, Kovacs G, Karydas A, Staffaroni A, Franca Resende E, Kim E, Hwang J, Ramos E, Wojta K, Pasquini L, Pang S, Spina S, Allen I, Kramer J, Miller B, Seeley W, Grinberg L
Brain Pathology 2021;31(2):267-282

Interactions between ALS-linked FUS and nucleoporins are associated with defects in the nucleocytoplasmic transport pathway

Lin Y, Kumar M, Ramesh N, Anderson E, Nguyen A, Kim B, Cheung S, McDonough J, Skarnes W, Lopez-Gonzalez R, Landers J, Fawzi N, Mackenzie I, Lee E, Nickerson J, Grunwald D, Pandey U, Bosco D
Nature Neuroscience 2021;24(8):1077-1088

Quantifying the phase separation property of chromatin-associated proteins under physiological conditions using an anti-1,6-hexanediol index

Shi M, You K, Chen T, Hou C, Liang Z, Liu M, Wang J, Wei T, Qin J, Chen Y, Zhang M, Li T
Genome Biology 2021;22(1)

Young-onset frontotemporal dementia with FUS pathology

Gowell M, Baker I, Ansorge O, Husain M
Practical Neurology 2021;21(2):149-152

Disruption of orbitofrontal-hypothalamic projections in a murine ALS model and in human patients

Bayer D, Antonucci S, Müller H, Saad R, Dupuis L, Rasche V, Böckers T, Ludolph A, Kassubek J, Roselli F
Translational Neurodegeneration 2021;10(1)

Frontotemporal Dementia: Correlations Between Psychiatric Symptoms and Pathology

Scarioni M, Gami‐Patel P, Timar Y, Seelaar H, van Swieten J, Rozemuller A, Dols A, Scarpini E, Galimberti D, Hoozemans J, Pijnenburg Y, Dijkstra A
Annals of Neurology 2020;87(6):950-961

Quantitative patterns of motor cortex proteinopathy across ALS genotypes

Nolan M, Scott C, Gamarallage M, Lunn D, Carpenter K, McDonough E, Meyer D, Kaanumalle S, Santamaria-Pang A, Turner M, Talbot K, Ansorge O
Acta Neuropathologica Communications 2020;8(1)

Factors associated with development and distribution of granular/fuzzy astrocytes in neurodegenerative diseases

Miki T, Yokota O, Haraguchi T, Ishizu H, Hasegawa M, Ishihara T, Ueno S, Takenoshita S, Terada S, Yamada N
Brain Pathology 2020;30(4):811-830

Spectrum of tau pathologies in Huntington's disease

Baskota S, Lopez O, Greenamyre J, Kofler J
Laboratory Investigation 2019;99(7):1068-1077

FUS-mediated regulation of acetylcholine receptor transcription at neuromuscular junctions is compromised in amyotrophic lateral sclerosis

Picchiarelli G, Demestre M, Zuko A, Been M, Higelin J, Dieterlé S, Goy M, Mallik M, Sellier C, Scekic-Zahirovic J, Zhang L, Rosenbohm A, Sijlmans C, Aly A, Mersmann S, Sanjuan-Ruiz I, Hübers A, Messaddeq N, Wagner M, van Bakel N, Boutillier A, Ludolph A, Lagier-Tourenne C, Boeckers T, Dupuis L, Storkebaum E
Nature Neuroscience 2019;22(11):1793-1805

Synaptic FUS Localization During Motoneuron Development and Its Accumulation in Human ALS Synapses

Deshpande D, Higelin J, Schoen M, Vomhof T, Boeckers T, Demestre M, Michaelis J
Frontiers in Cellular Neuroscience 2019;13

An aberrant phase transition of stress granules triggered by misfolded protein and prevented by chaperone function

Mateju D, Franzmann T, Patel A, Kopach A, Boczek E, Maharana S, Lee H, Carra S, Hyman A, Alberti S
The EMBO Journal 2017;36(12):1669-1687

Super-Resolution Microscopy Reveals Presynaptic Localization of the ALS/FTD Related Protein FUS in Hippocampal Neurons

Schoen M, Reichel J, Demestre M, Putz S, Deshpande D, Proepper C, Liebau S, Schmeisser M, Ludolph A, Michaelis J, Boeckers T
Frontiers in Cellular Neuroscience 2016;9

Chorea as a clinical feature of the basophilic inclusion body disease subtype of fused-in-sarcoma-associated frontotemporal lobar degeneration

Kawakami I, Kobayashi Z, Arai T, Yokota O, Nonaka T, Aoki N, Niizato K, Oshima K, Higashi S, Katsuse O, Hosokawa M, Hasegawa M, Akiyama H
Acta Neuropathologica Communications 2016;4(1)

Familial Behavioral Variant Frontotemporal Dementia Associated With Astrocyte-Predominant Tauopathy

Ferrer I, Legati A, García-Monco J, Gomez-Beldarrain M, Carmona M, Blanco R, Seeley W, Coppola G
Journal of Neuropathology & Experimental Neurology 2015;74(4):370-379

Neuropathologic analysis of Tyr69His TTR variant meningovascular amyloidosis with dementia

Ziskin J, Greicius M, Zhu W, Okumu A, Adams C, Plowey E
Acta Neuropathologica Communications 2015;3(1)

A truncating SOD1 mutation, p.Gly141X, is associated with clinical and pathologic heterogeneity, including frontotemporal lobar degeneration

Nakamura M, Bieniek K, Lin W, Graff-Radford N, Murray M, Castanedes-Casey M, Desaro P, Baker M, Rutherford N, Robertson J, Rademakers R, Dickson D, Boylan K
Acta Neuropathologica 2015;130(1):145-157

Amelioration of toxicity in neuronal models of amyotrophic lateral sclerosis by hUPF1

Barmada S, Ju S, Arjun A, Batarse A, Archbold H, Peisach D, Li X, Zhang Y, Tank E, Qiu H, Huang E, Ringe D, Petsko G, Finkbeiner S
Proceedings of the National Academy of Sciences 2015;112(25):7821-7826

Optineurin immunoreactivity in neuronal and glial intranuclear inclusions in adult-onset neuronal intranuclear inclusion disease.

Nakamura M, Murray ME, Lin WL, Kusaka H, Dickson DW
American journal of neurodegenerative disease 2014;3(2):93-102

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;2(1)

ALS-associated mutation FUS-R521C causes DNA damage and RNA splicing defects

Qiu H, Lee S, Shang Y, Wang W, Au K, Kamiya S, Barmada S, Finkbeiner S, Lui H, Carlton C, Tang A, Oldham M, Wang H, Shorter J, Filiano A, Roberson E, Tourtellotte W, Chen B, Tsai L, Huang E
Journal of Clinical Investigation 2014;124(3):981-999

Activity-dependent FUS dysregulation disrupts synaptic homeostasis

Sephton C, Tang A, Kulkarni A, West J, Brooks M, Stubblefield J, Liu Y, Zhang M, Green C, Huber K, Huang E, Herz J, Yu G
Proceedings of the National Academy of Sciences 2014;111(44)

Frontotemporal dementia–amyotrophic lateral sclerosis syndrome locus on chromosome 16p12.1–q12.2: genetic, clinical and neuropathological analysis

Dobson-Stone C, Luty A, Thompson E, Blumbergs P, Brooks W, Short C, Field C, Panegyres P, Hecker J, Solski J, Blair I, Fullerton J, Halliday G, Schofield P, Kwok J
Acta Neuropathologica 2013;125(4):523-533

Arginine methylation next to the PY-NLS modulates Transportin binding and nuclear import of FUS

Dormann D, Madl T, Valori C, Bentmann E, Tahirovic S, Abou-Ajram C, Kremmer E, Ansorge O, Mackenzie I, Neumann M, Haass C
The EMBO Journal 2012;31(22):4258-4275

Distinct clinical and pathological characteristics of frontotemporal dementia associated with C9ORF72 mutations

Snowden J, Rollinson S, Thompson J, Harris J, Stopford C, Richardson A, Jones M, Gerhard A, Davidson Y, Robinson A, Gibbons L, Hu Q, DuPlessis D, Neary D, Mann D, Pickering-Brown S
Brain 2012;135(3):693-708

Intracellular localization and splicing regulation of FUS/TLS are variably affected by amyotrophic lateral sclerosis-linked mutations

Kino Y, Washizu C, Aquilanti E, Okuno M, Kurosawa M, Yamada M, Doi H, Nukina N
Nucleic Acids Research 2011;39(7):2781-2798

FET proteins TAF15 and EWS are selective markers that distinguish FTLD with FUS pathology from amyotrophic lateral sclerosis with FUS mutations

Neumann M, Bentmann E, Dormann D, Jawaid A, DeJesus-Hernandez M, Ansorge O, Roeber S, Kretzschmar H, Munoz D, Kusaka H, Yokota O, Ang L, Bilbao J, Rademakers R, Haass C, Mackenzie I
Brain 2011;134(9):2595-2609

A comparative clinical, pathological, biochemical and genetic study of fused in sarcoma proteinopathies

Lashley T, Rohrer J, Bandopadhyay R, Fry C, Ahmed Z, Isaacs A, Brelstaff J, Borroni B, Warren J, Troakes C, King A, Al-Saraj S, Newcombe J, Quinn N, Ostergaard K, Schroder H, Bojsen-Moller M, Braendgaard H, Fox N, Rossor M, Lees A, Holton J, Revesz T
Brain 2011;134(9):2548-2564

Distribution and Pattern of Pathology in Subjects with Familial or Sporadic Late-Onset Cerebellar Ataxia as Assessed by p62/Sequestosome Immunohistochemistry

Pikkarainen M, Hartikainen P, Soininen H, Alafuzoff I
The Cerebellum 2011;10(4):720-731

FUS‐immunoreactive inclusions are a common feature in sporadic and non‐SOD1 familial amyotrophic lateral sclerosis

Deng H, Zhai H, Bigio E, 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;67(6):739-748

FUS pathology defines the majority of tau- and TDP-43-negative frontotemporal lobar degeneration

Urwin H, Josephs K, Rohrer J, Mackenzie I, Neumann M, Authier A, Seelaar H, Van Swieten J, Brown J, Johannsen P, Nielsen J, Holm I, Dickson D, Rademakers R, Graff-Radford N, Parisi J, Petersen R, Hatanpaa K, White III C, Weiner M, Geser F, Van Deerlin V, Trojanowski J, Miller B, Seeley W, van der Zee J, Kumar-Singh S, Engelborghs S, De Deyn P, Van Broeckhoven C, Bigio E, Deng H, Halliday G, Kril J, Munoz D, Mann D, Pickering-Brown S, Doodeman V, Adamson G, Ghazi-Noori S, Fisher E, Holton J, Revesz T, Rossor M, Collinge J, Mead S, Isaacs A
Acta Neuropathologica 2010;120(1):33-41

Frequency of ubiquitin and FUS-positive, TDP-43-negative frontotemporal lobar degeneration

Seelaar H, Klijnsma K, de Koning I, van der Lugt A, Chiu W, Azmani A, Rozemuller A, van Swieten J
Journal of Neurology 2009;257(5):747-753

A new subtype of frontotemporal lobar degeneration with FUS pathology

Neumann M, Rademakers R, Roeber S, Baker M, Kretzschmar H, Mackenzie I
Brain 2009;132(11):2922-2931

FUS pathology in basophilic inclusion body disease

Munoz D, Neumann M, Kusaka H, Yokota O, Ishihara K, Terada S, Kuroda S, Mackenzie I
Acta Neuropathologica 2009;118(5):617-627

Abundant FUS-immunoreactive pathology in neuronal intermediate filament inclusion disease

Neumann M, Roeber S, Kretzschmar H, Rademakers R, Baker M, Mackenzie I
Acta Neuropathologica 2009;118(5):605-616

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Enhanced validation

Submitted by: Atlas Antibodies (provider)
Enhanced method: Genetic validation
Main image
Experimental details: Western blot analysis in U-251MG cells transfected with control siRNA, target specific siRNA probe #1 and #2, using Anti-FUS antibody. Remaining relative intensity is presented. Loading control: Anti-GAPDH.
Sample type: Human
Protocol: Protocol

Supportive validation

Submitted by: Atlas Antibodies (provider)
Enhanced method: Orthogonal validation
Main image
Experimental details: Immunohistochemistry analysis in human endometrium and liver tissues using HPA008784 antibody. Corresponding FUS RNA-seq data are presented for the same tissues.
Sample type: Human
Protocol: Protocol
Orthogonal method: Orthogonal validation of protein expression using IHC by comparison to RNA-seq data of corresponding target in high and low expression tissues.

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