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| Alternative Name : |
Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3, AT3, JOS. |
| Amount : |
10 µg |
Source : Escherichia Coli. ATXN3 produced in E.Coli is a single, non-glycosylated polypeptide chain containing 370 amino acids (1-370 a.a.) and having a molecular mass of 42.4kDa.ATXN3 is purified by proprietary chromatographic techniques. Ataxin 3 is otherwise known as Machado-Joseph disease protein 1. Machado-Joseph disease is a hereditary autosomal dominant neurodegenerative disorder. ATXN3 contains trinucleotide CAG repeats in the coding region, and the expansion of these repeats from the normal 13-36 to 68-79 causes the Machado-Joseph disease. ATXN3 is a poly-ubiquitin-binding protein whose cellular turnover is regulated by its catalytic activity.In addition, ATXN3 is a proteasome-associated factor which mediates the degradation of ubiquitinated proteins. ATXN3 folds reversibly using a single intermediate; partial destabilization of ATXN3 by chemical denaturation causes the formation of fibrillar aggregates by the non-pathological variant.Ataxin-3 interacts with the major histone acetyltransferases cAMP-response-element binding protein (CREB)-binding protein, p300, and p300/CREB-binding protein-associated factor and hinders transcription by these coactivators.
