Active Recombinant Human ATXN3, GST-tagged

• Cat.No.: ATXN3-173H
• Product Overview: Recombinant human ATXN3 (amino acid residues 1-370), fused with N-terminal GST, was expressed in E.coli.

Specification

• Description: There are two main classes of DUB; cysteine proteases and metalloproteases. Ataxin-3 is a cysteine protease and is a member of the Machado-Joseph Domain (MJD) enzyme family. Cloning of the human gene was first described by Kawaguchi et al. (1994). Machado–Joseph disease (MJD), the most common form of spinocerebellar ataxia worldwide, is a progressive and ultimately fatal neurodegenerative disorder caused by polyQ expansion in ataxin-3, a conserved and ubiquitous protein known to bind polyubiquitin chains and to function as a deubiquitylating enzyme. Ataxin-3 has been linked to protein homeostasis maintenance, transcription, cytoskeleton regulation and myogenesis (Matos et al., 2011).
• Source: E. coli
• Species: Human
• Tag: GST
• Form: 50 mM HEPES pH 7.5, 150 mM sodium chloride, 2 mM dithiothreitol, 10% glycerol
• Bio-activity: Deubiquitylase Enzyme Assay: The activity of GST-Ataxin-3 was validated by determining the increase in fluorescence measured as a result of the enzyme catalysed cleavage of the fluorogenic substrate Ubiquitin-Rhodamine110-Glycine generating Ubiquitin and Rhodamine110-Glycine. Incubation of the substrate in the presence or absence of GST-Ataxin-3 was compared confirming the deubiquitylating activity of GST-Ataxin-3.
• Molecular Mass: ~69 kDa
• Purity: >98% by SDS-PAGE
• Storage: 12 months at -70°C. Avoid multiple freeze/thaw cycles.
• Concentration: 0.5 mg/ml

Gene Information

• Gene Name: ATXN3 ataxin 3 [ Homo sapiens ]
• Official Symbol: ATXN3
• Synonyms: ATXN3; ataxin 3; Machado Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3) , MJD, SCA3; ataxin-3; ATX3; JOS; josephin; ataxin 3 variant h; ataxin 3 variant m; ataxin 3 variant ref; olivopontocerebellar ataxia 3; Machado-Joseph disease protein 1; spinocerebellar ataxia type 3 protein; Machado-Joseph disease (spinocerebellar ataxia 3, olivopontocerebellar ataxia 3, autosomal dominant, ataxin 3); AT3; MJD; MJD1; SCA3
• Gene ID: 4287
• mRNA Refseq: NM_001127696
• Protein Refseq: NP_001121168
• MIM: 607047
• UniProt ID: P54252
• Chromosome Location: 14q21
• Pathway: Protein processing in endoplasmic reticulum, organism-specific biosystem; Protein processing in endoplasmic reticulum, conserved biosystem
• Function: cysteine-type peptidase activity; omega peptidase activity; peptidase activity; protein binding