human, mouse, rat, bovine, canine, chicken/avian, donkey, feline, goat, guinea pig, hamster, horse, porcine, rabbit, sheep, simian, other
Isotype
n/a
Format
immunoaffinity purified
Size
1.5 mg
Concentration
3 mg/ml
Applications
WB
Reviews / Ratings
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Description
Goat polyclonal antibody to ATXN3. This protein appears to be a component of the ubiquitin proteasome system and has deubiquitinase activity. It may also have roles in neuroprotection, protein homeostasis maintenance, transcriptional and cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates. The protein contains Qn repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 is one cause of Machado-Joseph disease also known as spinocerebellar ataxia-3, an autosomal dominant neurologic disorder. Detects levels of ATXN3 protein in transfected cells with GFP-ATXN3 and endogenous levels in U118, SH-SY5Y, At-T20 and hTERT cell lysates by Western blot.
Immunogen
Purified recombinant peptide derived from within residues 120 aa to 250 aa of human ATXN3 produced in E. coli.
Other Names
AT3, ATX3, ataxin-3, ataxin 3 variant h, autosomal dominant ataxin 3, ataxin 3 variant m, ataxin 3 variant ref, JOS, josephin, Machado-Joseph, Machado-Joseph disease protein 1, MJD, MJD1, SCA3, spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, spinocerebellar ataxia type 3 protein