This Antibody was verified by Knockout to ensure that the antibody binds to the antigen stated. View Details
Recommended positive controls: 293T, A431, HeLa, HepG2, SK-N-SH, SK-N-AS, human Ataxin3-transfected 293T.
Predicted reactivity: Mouse (88%), Rat (82%), Pig (89%), Sheep (92%), Bovine (93%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
Machado-Joseph disease is an autosomal dominant neurologic disorder, and is now known to be the same as previously described spinocerebellar ataxia-3. MJD protein (Ataxin-3) contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is the cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. This protein interacts with key regulators (CBP, p300 and PCAF) of transcription and represses transcription, and also acts as a histone-binding protein that regulates transcription. MJD is a deubiquitinating enzyme.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: ataxin 3 variant an; ataxin 3 variant ao; ataxin 3 variant at; ataxin 3 variant e; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant r; ataxin 3 variant ref; ataxin 3 variant y; Ataxin-3; ataxin3; josephin; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) homolog; Machado-Joseph disease protein 1; Machado-Joseph disease protein 1 homolog; olivopontocerebellar ataxia 3; spin; Spinocerebellar ataxia type 3 protein
Gene Aliases: 2210008M02Rik; AI463012; AI647473; AT3; ataxin-3; ATX3; ATXN3; JOS; MJD; MJD1; SCA3
Molecular Function: transcription factor