Analysis Note

ControlHuman SCA-3/MJD brain sections, NIH/3T3 lysate

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.

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.

Immunogen

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

Legal Information

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

Other Notes

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

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.

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).

Target description

44 kDa