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MAB5360

Sigma-Aldrich

Anti-Spinocerebellar Ataxia Type 3 Antibody, clone 1H9

ascites fluid, clone 1H9, Chemicon®

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Synonym(s):
Ataxin-3, josephin
eCl@ss:
32160702
NACRES:
NA.41

biological source

mouse

Quality Level

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.

UniProt accession no.

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

Storage Class

10 - Combustible liquids

wgk_germany

nwg

flash_point_f

Not applicable

flash_point_c

Not applicable


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