His6-Ataxin-3

PDF

E-341

50 μg

Brand

Boston Biochem

Description

His6-Ataxin-3

Catalog #: E-341

His6-Ataxin-3
Product Source: 

human recombinant

His6-Ataxin-3 (MJD protein 1),

Defects in the ATXN3 gene are the cause of Machado-Joseph disease (MJD), also known as spinorerebellar ataxia 3. MJD is an autosomal dominant neurodegenerative disorder caused by an expansion of a (CAG)n repeat in the ATXN3 gene resulting in polymorphic protein forms in the C-terminal poly-glutamine region. Ataxin-3 protein belongs to a novel group of cysteine proteases similar to USP-type ubiquitin proteases and has deubiquitinating activity in vitro. The full-length protein contains an N-terminal Josephin domain, two ubiquitin interacting motifs (UIMs), and a variable C-terminus consisting of a polyglutamine stretch and tail. Ataxin-3 activity may have a role in MJD pathogenesis by influencing ubiquitin-dependent pathways that control protein folding and stability. This recombinant protein is N-terminally tagged. (Accession # NP_004984).

Product Information
Quantity: 

50 μg

Stock: 

X mg/ml (X μM) in 50 mM HEPES pH 8.0, 100 mM NaCl, 10% glycerol, 0.5 mM EDTA, 1mM DTT. Actual concentration varies with lot number.

MW: 

42 kDa

Purity: 

> 95% by SDS-PAGE

 

Use & Storage
Use: 

Typical enzyme concentration for use in vitro ranges from 1-5 μM depending on conditions and substrate.

Storage: 

Store at -80°C. Avoid multiple freeze/thaw cycles.

Application

Reactivity

Photos

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