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MAB5360

Sigma-Aldrich

Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9

ascites fluid, clone 1H9, Chemicon®

Synonym(s):

Ataxin-3, josephin

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About This Item

UNSPSC Code:
12352203
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

100

antibody form

ascites fluid

antibody product type

primary antibodies

clone

1H9, monoclonal

species reactivity

rat, human, monkey, mouse

manufacturer/tradename

Chemicon®

technique(s)

ELISA: suitable
immunocytochemistry: suitable
immunohistochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable

isotype

IgG1

NCBI accession no.

NM_001024631.1

UniProt accession no.

P54252

shipped in

dry ice

target post-translational modification

unmodified

Gene Information

human ... ATXN3(4287)

General description

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.

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).

Immunogen

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

Application

Detect Spinocerebellar Ataxia Type 3 using this Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9 validated for use in ELISA, IC, IH, IP & WB.
Immunohistochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IH.

Immunoprecipitation:
A 1:500-1:5000 dilution of a previous lot was used in IP.

ELISA:
A 1:500-1:5000 dilution of a previous lot was used in ELISA.

Immunocytochemistry:
A 1:500-1:5000 dilution of a previous lot was used in IC.

Optimal working dilutions must be determined by the end user.

Quality

Evaluated by Western Blot on NIH/3T3 lysates.

Western Blot Analysis:
1:500 dilution of this antibody detected SPINOCEREBELLAR ATAXIA 3 on 10 µg of NIH/3T3 lysates.

Target description

44 kDa

Analysis Note

Control
Human SCA-3/MJD brain sections, NIH/3T3 lysate

Other Notes

Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.

Legal Information

CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany

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Storage Class Code

10 - Combustible liquids

WGK

nwg

Flash Point(F)

Not applicable

Flash Point(C)

Not applicable

Regulatory Listings

Regulatory Listings are mainly provided for chemical products. Only limited information can be provided here for non-chemical products. No entry means none of the components are listed. It is the user’s obligation to ensure the safe and legal use of the product.

JAN Code

MAB5360:

Certificates of Analysis (COA)

Search for Certificates of Analysis (COA) by entering the products Lot/Batch Number. Lot and Batch Numbers can be found on a product’s label following the words ‘Lot’ or ‘Batch’.

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Y Trottier et al.
Neurobiology of disease, 5(5), 335-347 (1999-03-09)
Spinocerebellar ataxia type 3 or Machado-Joseph disease (SCA3/MJD) is an autosomal dominant neurodegenerative disorder caused by an unstable and expanded CAG trinucleotide repeat that leads to the expansion of a polyglutamine tract in a protein of unknown function, ataxin-3. We
Christopher P Reina et al.
Cell stress & chaperones, 17(6), 729-742 (2012-07-11)
Regulation of basal and induced levels of hsp70 is critical for cellular homeostasis. Ataxin-3 is a deubiquitinase with several cellular functions including transcriptional regulation and maintenance of protein homeostasis. While investigating potential roles of ataxin-3 in response to cellular stress
Overexpression of Mutant Ataxin-3 in Mouse Cerebellum Induces Ataxia and Cerebellar Neuropathology.
Nobrega, Clevio, et al.
Cerebellum (2012)
Allele-selective inhibition of ataxin-3 (ATX3) expression by antisense oligomers and duplex RNAs.
Hu, J; Gagnon, KT; Liu, J; Watts, JK; Syeda-Nawaz, J; Bennett, CF; Swayze, EE; Randolph et al.
Biological Chemistry null
Fiona M Menzies et al.
Brain : a journal of neurology, 133(Pt 1), 93-104 (2009-12-17)
Spinocerebellar ataxia type 3 is a neurodegenerative disorder caused by the expansion of the polyglutamine repeat region within the ataxin-3 protein. The mutant protein forms intracellular aggregates in the brain. However, the cellular mechanisms causing toxicity are still poorly understood
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