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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.
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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 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;

For Research Use Only. Not intended for any clinical use. No products from Creative BioMart may be resold, modified for resale or used to manufacture commercial products without prior written approval from Creative BioMart.

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Q&As (6)

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What are the clinical manifestations of SCA3? 10/15/2022

The clinical manifestations of SCA3 include ataxia (especially limb motor coordination disorder), myoclonus, ocular muscle movement abnormalities, extrapyramidal symptoms, dysphagia, language disorder, etc. As the disease progresses, patients have more and more significant difficulties with walking, balance, and other movements.

Which cellular processes are ATXN3 proteins involved in? 02/23/2022

ATXN3 protein is involved in many important cellular processes, including protein degradation, cell signaling, apoptosis, RNA metabolism, etc. It plays an important role in the regulation of cellular functions.

Is there any treatment for SCA3? 07/09/2021

At present, there is no cure for SCA3, but some adjuvant therapies can be used to improve symptoms. Physical therapy, speech therapy and swallowing function training can improve the quality of life of patients. In addition, there are some studies on protein degradation pathways for abnormal aggregation of ATXN3 protein, but they are still in the experimental stage.

Is ATXN3 protein associated with other diseases? 03/23/2021

In addition to its association with SCA3, ATXN3 protein may also be associated with some other diseases. For example, studies have shown that ATXN3 protein is related to the occurrence and development of neurodegenerative diseases such as Parkinson's disease and Huntington's disease, but the specific mechanism needs to be further studied and verified.

How do ATXN3 protein abnormalities cause SCA3? 07/06/2020

SCA3 is caused by mutations in the ATXN3 gene that lead to excessive repeat of polyglutamine (PolyQ) sequences in ATXN3 protein. This abnormal expansion will cause abnormal ATXN3 protein folding and aggregation, leading to impaired cell function and eventually triggering SCA3 symptoms.

Which disease is ATXN3 protein associated with? 11/26/2019

ATXN3 protein is associated with autodominant genetic disease Spinocerebellar Ataxia Type 3 (SCA3). SCA3 is a neurodegenerative disease characterized by ataxia, extrapyramidal symptoms, and motor neuron dysfunction.

Customer Reviews (3)

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Reviews
06/21/2021

    ATXN3 is very catalytic active and shows excellent catalytic performance even at low concentrations.

    08/14/2019

      ATXN3 detection method is sensitive and can detect trace amounts of protein, which is well suited for our study.

      03/25/2019

        Using ATXN3, the reproducibility of my experiments is very good, which facilitates subsequent data analysis and experimental manipulation.

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