Material Data Sheet

Ataxin-3, His6 tag

Price: $119.00
Catalog #: E-341

Ataxin-3, His6 tag

50 µg
Data Sheet: 

Ataxin-3, also known as Machado Joseph Disease (MJD) Protein 1 and Spinocerebellar Ataxia Type 3, is a 364 amino acid (aa), ubiquitously expressed cytoplasmic and nuclear protein with a predicted molecular weight of 42 kDa. Ataxin-3 functions as a deubiquitinating enzyme, and shares 87% and 86% aa sequence identity with mouse and rat Ataxin-3, respectively.   Full-length Ataxin-3 contains an N-terminal Josephin domain, two Ubiquitin interacting motifs (UIM’s), and a variable C-terminus consisting of a polyglutamine stretch and tail. As a deubiquitinating enzyme, Ataxin-3 plays a critical role in affecting the ubiquitination status of proteins for quality control and other cellular pathways. In turn, the ubiquitination of Ataxin-3 was shown to enhance its capacity to cleave Ubiquitin chains.  By opposing the actions of the Ubiquitin-conjugating (E2) enzyme UBE2W, Ataxin-3 is believed to control the activity of the Ubiquitin E3 ligase named CHIP (aka STUB1). CHIP binds to protein chaperones and represents an important molecular link between the chaperone and Ubiquitin-Proteasome system.  Mutations in the ATXN3 gene are the cause of MJD, also known as spinocerebellar ataxia 3, an autosomal dominant neurodegenerative disorder characterized by nuclear aggregation of Ataxin-3 molecules featuring an expanded polyglutamine tract.  This recombinant protein contains an N-terminal 6His tag.

Product Information

X mg/ml (X μM) in 50 mM HEPES pH 8.0, 100 mM NaCl, 10% (v/v) Glycerol, 0.5 mM EDTA, 1 mM DTT
42 kDa
> 95% by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie Blue stain

Use & Storage


Recombinant Human His6-Ataxin-3 is a Ubiquitin-specific deconjugating enzyme.  Reaction conditions will need to be optimized for each specific application. We recommend an initial  Ataxin-3 concentration of 10-100 nM when using K63-linked tetra-Ubiquitin Rhodamine (UC-355) as a substrate. Higher enzyme concentrations may be required for other substrates.


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