ATXN3 Human

Ataxin-3 Human Recombinant
Cat. No.
BT26046
Source
Escherichia Coli.
Synonyms
Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3, AT3, JOS.
Appearance
Sterile filtered colorless solution.
Purity
Greater than 90.0% as determined by SDS-PAGE.
Usage
THE BioTek's products are furnished for LABORATORY RESEARCH USE ONLY. The product may not be used as drugs, agricultural or pesticidal products, food additives or household chemicals.
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Description

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.

Product Specs

Introduction
Ataxin 3, also referred to as Machado-Joseph disease protein 1, is associated with the autosomal dominant neurodegenerative disorder known as Machado-Joseph disease. This protein, ATXN3, contains CAG trinucleotide repeats within its coding region. An abnormal expansion of these repeats, from the typical range of 13-36 to 68-79, leads to the development of Machado-Joseph disease. Functionally, ATXN3 acts as a poly-ubiquitin-binding protein, with its cellular turnover being regulated by its own catalytic activity. Moreover, ATXN3 plays a role in the proteasome pathway, acting as a factor that facilitates the degradation of proteins tagged with ubiquitin. The protein exhibits reversible folding through a single intermediate state. Interestingly, even the non-pathological variant of ATXN3 can form fibrillar aggregates when partially destabilized, such as through chemical denaturation. In terms of interactions, Ataxin-3 binds to key histone acetyltransferases, including cAMP-response-element binding protein (CREB)-binding protein (CBP), p300, and p300/CREB-binding protein-associated factor (PCAF), ultimately hindering the transcriptional activity of these coactivators.
Description
Recombinant ATXN3, expressed in E. coli, is a single, non-glycosylated polypeptide chain. It encompasses amino acids 1 to 370, resulting in a molecular weight of 42.4 kDa. The purification of ATXN3 is achieved using specialized chromatographic techniques.
Physical Appearance
A clear, colorless solution that has been sterilized by filtration.
Formulation
The ATXN3 protein solution is supplied in a buffer consisting of 20mM Tris-HCl (pH 7.5), 2mM DTT, 50mM NaCl, and 10% glycerol.
Stability
For short-term storage (up to 2-4 weeks), the ATXN3 protein solution should be kept at 4°C. For longer storage, it is recommended to freeze the solution at -20°C. To further enhance stability during long-term storage, consider adding a carrier protein like HSA or BSA (0.1%). It is important to minimize repeated freeze-thaw cycles to maintain protein integrity.
Purity
The purity of ATXN3 is determined by SDS-PAGE analysis and is consistently found to be greater than 90%.
Synonyms
Ataxin-3, Machado-Joseph disease protein 1, Spinocerebellar ataxia type 3 protein, ATXN3, ATX3, MJD, MJD1, SCA3, AT3, JOS.
Source
Escherichia Coli.
Amino Acid Sequence
MESIFHEKQE GSLCAQHCLN NLLQGEYFSP VELSSIAHQL DEEERMRMAE GGVTSEDYRT FLQQPSGNMD DSGFFSIQVI SNALKVWGLELILFNSPEYQ RLRIDPINER SFICNYKEHW FTVRKLGKQW FNLNSLLTGP ELISDTYLAL FLAQLQQEGY SIFVVKGDLP DCEADQLLQM IRVQQMHRPK LIGEELAQLK EQRVHKTDLE RVLEANDGSG MLDEDEEDLQ RALALSRQEI DMEDEEADLR RAIQLSMQGS SRNISQDMTQ TSGTNLTSEE LRKRREAYFE KQQQKQQQQQ QQQQQQQQQQ QQQQGDLSGQ SSHPCERPAT SSGALGSDLG DAMSEEDMLQ AAVTMSLETV RNDLKTEGKK.

Product Science Overview

Structure and Function

Ataxin-3 is a deubiquitinating enzyme (DUB) that has two distinct features:

  1. Josephin Domain: This N-terminal domain confers cysteine protease activity to Ataxin-3, which is essential for hydrolyzing ubiquitin (Ub) linkages.
  2. Ubiquitin-Interacting Motifs (UIMs): Ataxin-3 contains three UIMs that allow it to bind Ub conjugates and ubiquitinated proteins, bringing them into proximity to trim or edit specific linkages within these Ub conjugates .

Ataxin-3 binds long poly-Ub chains and trims them, but it has weak or no activity against chains of four or fewer Ub .

Clinical Significance

Ataxin-3 is most notably associated with Machado-Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3). This autosomal dominant neurologic disorder is caused by the expansion of CAG repeats in the coding region of the ATXN3 gene. The normal range of CAG repeats is 13-36, but in individuals with MJD, this range expands to 68-79 repeats . This disorder is classified as a polyglutamine (PolyQ) disease, and there is an inverse correlation between the age of onset and the number of CAG repeats .

Recombinant Ataxin-3

Recombinant human Ataxin-3 is typically expressed in E. coli and purified for research purposes. It is often tagged with His tags to facilitate purification and detection. The recombinant protein retains the functional domains of the native protein, making it useful for studying the biochemical properties and interactions of Ataxin-3 .

Interactions

Ataxin-3 has been shown to interact with several proteins, including:

  • RAD23A and RAD23B: These are human homologs of yeast DNA repair proteins .
  • Valosin-Containing Protein (VCP): This interaction is consistent with Ataxin-3’s role in ubiquitin-mediated proteolysis .
Research Applications

Recombinant Ataxin-3 is used in various research applications to study its role in neurodegenerative diseases, protein degradation pathways, and cellular stress responses. It is also valuable for investigating potential therapeutic targets for treating disorders like Machado-Joseph disease.

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