Ataxin-3 | Machado-Joseph disease protein 1 | Spinocerebellar ataxia type 3 protein | ATXN3 | ATX3 | MJD | MJD1 | SCA3 | AT3 | JOS
规格或纯度
无载体,生物活性,ActiBioPure™,His标签,≥95%(SDS-PAGE),见COA
生化机理
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.
Post-translational: Monoubiquitinated N-terminally by UBE2W, possibly leading to activate the deubiquitinating enzyme activity (PubMed:23696636).
生物活性
Immobilized Recombinant Human Ataxin 3 Protein (rp184114) at 1.0 μg/mL can bind Recombinant Ataxin 3 Antibody (Ab089900) with the EC50 of 2.1 ng/mL.
Store at -20°C for 1 year. Avoid freeze/thaw cycle.
图片
Recombinant Human Ataxin 3 Protein (rp184114) - ELISA Immobilized Recombinant Human Ataxin 3 Protein (rp184114) at 1.0 μg/mL can bind Recombinant Ataxin 3 Antibody (Ab089900) with the EC50 of 2.1 ng/mL.
Recombinant Human Ataxin 3 Protein (rp184114) - SDS-PAGE 3 μg/lane of Recombinant Human Ataxin 3 Protein was resolved with SDS-PAGE under reducing (R) and non-reducing (N) conditions and visualized by Coomassie® Blue staining, showing a band at 43.3 kDa.