Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 | EMD Millipore MAB5360 product information

This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.

product summary

company name :

EMD Millipore

other brands :

Oncogene Research Products, Calbiochem, Novagen, Merck, Upstate Biotechnology, Chemicon, LINCO, Novabiochem, Guava

product type :

antibody

product name :

Spinocerebellar Ataxia Type 3 Antibody, clone 1H9

catalog :

MAB5360

quantity :

100 µL

clonality :

monoclonal

host :

mouse

conjugate :

nonconjugated

clone name :

1H9

reactivity :

African green monkey, human, mouse, rat

application :

western blot, ELISA, immunohistochemistry, immunocytochemistry, immunoprecipitation, immunohistochemistry - paraffin section, immunohistochemistry - free floating section, western blot knockout validation

citations: 31

Published Application/Species/Sample/Dilution	Reference
western blot; human; loading ...; fig S2A western blot knockout validation; mouse; loading ...; fig 2A	Tu Y, Liu H, Zhu X, Shen H, Ma X, Wang F, et al. Ataxin-3 promotes genome integrity by stabilizing Chk1. Nucleic Acids Res. 2017;45:4532-4549 pubmed publisher
western blot knockout validation; mouse; 1:20,000; fig 2	Reina C, Nabet B, Young P, Pittman R. Basal and stress-induced Hsp70 are modulated by ataxin-3. Cell Stress Chaperones. 2012;17:729-42 pubmed publisher
western blot; human; loading ...; fig ev1f	Singh A, Oehler J, Torrecilla I, Kilgas S, Li S, Vaz B, et al. The p97-Ataxin 3 complex regulates homeostasis of the DNA damage response E3 ubiquitin ligase RNF8. EMBO J. 2019;38:e102361 pubmed publisher
western blot; mouse; 1:1000; loading ...; fig 4b	Sowa A, Martin E, Martins I, Schmidt J, Depping R, Weber J, et al. Karyopherin ?-3 is a key protein in the pathogenesis of spinocerebellar ataxia type 3 controlling the nuclear localization of ataxin-3. Proc Natl Acad Sci U S A. 2018;115:E2624-E2633 pubmed publisher
immunoprecipitation; human; loading ...; fig 2a western blot; human; loading ...; fig 4b western blot; mouse; loading ...; fig 1a	Ashkenazi A, Bento C, Ricketts T, Vicinanza M, Siddiqi F, Pavel M, et al. Polyglutamine tracts regulate beclin 1-dependent autophagy. Nature. 2017;545:108-111 pubmed publisher
immunocytochemistry; human; 1:1000; loading ...; tbl s4 western blot; human; 1:1000; loading ...; tbl s4	Hansen S, Stummann T, Borland H, Hasholt L, Tumer Z, Nielsen J, et al. Induced pluripotent stem cell - derived neurons for the study of spinocerebellar ataxia type 3. Stem Cell Res. 2016;17:306-317 pubmed publisher
western blot; human; 1:1000; loading ...; tbl 2	Hansen S, Borland H, Hasholt L, Tumer Z, Nielsen J, Rasmussen M, et al. Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.B11. Stem Cell Res. 2016;16:589-92 pubmed publisher
western blot; human; 1:1000; loading ...; fig 1f	Hansen S, Borland H, Hasholt L, Tumer Z, Nielsen J, Rasmussen M, et al. Generation of spinocerebellar ataxia type 3 patient-derived induced pluripotent stem cell line SCA3.A11. Stem Cell Res. 2016;16:553-6 pubmed publisher
western blot; human; 1:1000; loading ...; fig 2d	Matos C, NÃ³brega C, Louros S, Almeida B, Ferreiro E, Valero J, et al. Ataxin-3 phosphorylation decreases neuronal defects in spinocerebellar ataxia type 3 models. J Cell Biol. 2016;212:465-80 pubmed publisher
immunohistochemistry - free floating section; mouse; 1:1000 immunohistochemistry - paraffin section; human; 1:200 immunoprecipitation; human western blot; human; 1:1000	Zeng L, Wang B, Merillat S, Minakawa E, Perkins M, Ramani B, et al. Differential recruitment of UBQLN2 to nuclear inclusions in the polyglutamine diseases HD and SCA3. Neurobiol Dis. 2015;82:281-288 pubmed publisher
western blot; African green monkey; 1:10,000; loading ...; fig 1	Almeida B, Abreu I, Matos C, Fraga J, Fernandes S, Macedo M, et al. SUMOylation of the brain-predominant Ataxin-3 isoform modulates its interaction with p97. Biochim Biophys Acta. 2015;1852:1950-9 pubmed publisher
western blot; mouse; 1:500; fig 2	Yao Y, Cui X, Al Ramahi I, Sun X, Li B, Hou J, et al. A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity. elife. 2015;4: pubmed publisher
western blot; human; loading ...; fig 3a	Gao R, Liu Y, Silva Fernandes A, Fang X, Paulucci Holthauzen A, Chatterjee A, et al. Inactivation of PNKP by mutant ATXN3 triggers apoptosis by activating the DNA damage-response pathway in SCA3. PLoS Genet. 2015;11:e1004834 pubmed publisher
immunocytochemistry; rat; 1:300 western blot; human; 1:1000	Liman J, Deeg S, Voigt A, Vo feldt H, Dohm C, Karch A, et al. CDK5 protects from caspase-induced Ataxin-3 cleavage and neurodegeneration. J Neurochem. 2014;129:1013-23 pubmed publisher
western blot; human	Aiba Y, Hu J, Liu J, Xiang Q, Martinez C, Corey D. Allele-selective inhibition of expression of huntingtin and ataxin-3 by RNA duplexes containing unlocked nucleic acid substitutions. Biochemistry. 2013;52:9329-38 pubmed publisher
western blot; human	Liu J, Yu D, Aiba Y, Pendergraff H, Swayze E, Lima W, et al. ss-siRNAs allele selectively inhibit ataxin-3 expression: multiple mechanisms for an alternative gene silencing strategy. Nucleic Acids Res. 2013;41:9570-83 pubmed publisher
western blot; human	Liu J, Pendergraff H, Narayanannair K, Lackey J, Kuchimanchi S, Rajeev K, et al. RNA duplexes with abasic substitutions are potent and allele-selective inhibitors of huntingtin and ataxin-3 expression. Nucleic Acids Res. 2013;41:8788-801 pubmed publisher
immunohistochemistry; mouse; 1:4000 western blot; mouse; 1:2500	Nguyen H, Hübener J, Weber J, Grueninger S, Riess O, Weiss A. Cerebellar soluble mutant ataxin-3 level decreases during disease progression in Spinocerebellar Ataxia Type 3 mice. PLoS ONE. 2013;8:e62043 pubmed publisher
western blot; human; 1:4000 immunohistochemistry; mouse; 1:1000	Hübener J, Weber J, Richter C, Honold L, Weiss A, Murad F, et al. Calpain-mediated ataxin-3 cleavage in the molecular pathogenesis of spinocerebellar ataxia type 3 (SCA3). Hum Mol Genet. 2013;22:508-18 pubmed publisher
	Zou H, Chen H, Zhou Z, Wan Y, Liu Z. ATXN3 promotes breast cancer metastasis by deubiquitinating KLF4. Cancer Lett. 2019;467:19-28 pubmed publisher
	Gao R, Chakraborty A, Geater C, Pradhan S, Gordon K, Snowden J, et al. Mutant huntingtin impairs PNKP and ATXN3, disrupting DNA repair and transcription. elife. 2019;8: pubmed publisher
	Liu J, Liu Z, Corey D. The Requirement for GW182 Scaffolding Protein Depends on Whether Argonaute Is Mediating Translation, Transcription, or Splicing. Biochemistry. 2018;57:5247-5256 pubmed publisher
	Koyuncu S, Saez I, Lee H, Gutiérrez García R, Pokrzywa W, Fatima A, et al. The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington's disease patients. Nat Commun. 2018;9:2886 pubmed publisher
	Esteves S, Duarte Silva S, Naia L, Neves Carvalho A, Teixeira Castro A, Rego A, et al. Limited Effect of Chronic Valproic Acid Treatment in a Mouse Model of Machado-Joseph Disease. PLoS ONE. 2015;10:e0141610 pubmed publisher
	Sun X, Li P, Zhu S, Cohen R, Marque L, Ross C, et al. Nuclear retention of full-length HTT RNA is mediated by splicing factors MBNL1 and U2AF65. Sci Rep. 2015;5:12521 pubmed publisher
	Fiszer A, Olejniczak M, Switonski P, Wroblewska J, Wisniewska Kruk J, Mykowska A, et al. An evaluation of oligonucleotide-based therapeutic strategies for polyQ diseases. BMC Mol Biol. 2012;13:6 pubmed publisher
	Hübener J, Vauti F, Funke C, Wolburg H, Ye Y, Schmidt T, et al. N-terminal ataxin-3 causes neurological symptoms with inclusions, endoplasmic reticulum stress and ribosomal dislocation. Brain. 2011;134:1925-42 pubmed publisher
	Seidel K, den Dunnen W, Schultz C, Paulson H, Frank S, de Vos R, et al. Axonal inclusions in spinocerebellar ataxia type 3. Acta Neuropathol. 2010;120:449-60 pubmed publisher
	Menzies F, Huebener J, Renna M, Bonin M, Riess O, Rubinsztein D. Autophagy induction reduces mutant ataxin-3 levels and toxicity in a mouse model of spinocerebellar ataxia type 3. Brain. 2010;133:93-104 pubmed publisher
	Hu J, Matsui M, Gagnon K, Schwartz J, Gabillet S, Arar K, et al. Allele-specific silencing of mutant huntingtin and ataxin-3 genes by targeting expanded CAG repeats in mRNAs. Nat Biotechnol. 2009;27:478-84 pubmed publisher
	Schmitt I, Linden M, Khazneh H, Evert B, Breuer P, Klockgether T, et al. Inactivation of the mouse Atxn3 (ataxin-3) gene increases protein ubiquitination. Biochem Biophys Res Commun. 2007;362:734-9 pubmed

product information

Catalog Number :

MAB5360

Subcategory :

Neuroscience

Product Name :

Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9

Product Type :

Antibodies

Clonality :

Monoclonal Antibody

Gene ID :

P54252

Host Name :

Mouse

Antigen :

Spinocerebellar Ataxia Type 3

Clone :

1H9

Conjugate :

Ascites

Isotype :

IgG1

Product Description :

Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9

Cross Reactivity :

Human;Mouse;Rat;Monkey

Background :

Spinocerebellar ataxia (SCA) is a genetic disease with multiple types, each of which could be considered a disease in its own right. Spinocerebellar ataxia (SCA) is one of a group of genetic disorders characterized by slowly progressive incoordination of gait and often associated with poor coordination of hands, speech, and eye movements. Frequently, atrophy of the cerebellum occurs. The first ataxia gene was identified in 1993 for a dominantly inherited type called “Spinocerebellar ataxia type 1" (SCA1). Subsequently, as additional dominant genes were found they were called SCA2, SCA3, etc. Usually, the "type" number of "SCA" refers to the order in which the gene was found. At this time, there are at least 29 different gene mutations which have been found.

ALT Names :

Ataxin-3;josephin

Immunogen :

Human ataxin-3 fragment from aa F112-L249 as a fusion protein

Specificity :

Ataxin-3. The epitope was mapped precisely at E214-L233. MAB5360 can be used to study wild type ataxin-3 and the mutant form with polyglutamine expansion found in patients affected with spinocerebellar ataxin type 3/Machado-Joseph disease (SCA3/MJD). In analysis of human tissues by Western blot, MAB5360 releaved several isoforms of ataxin-3 (presumably generated by alternative splicing, Trottier et al. 1998). The antibody detected polyglutamine aggregate (or nuclear inclusions) by IHC on SCA-3/MJD brain sections (Paulson et al. 1997).

Package Size :

100 µL

Uses :

ELISA;Immunocytochemistry;Immunohistochemistry;Immunoprecipitation;Western Blotting

Storage :

Stable for 1 year at -20ºC from date of receipt.