Full Product Name
ATX3 Polyclonal Antibody
Product Synonym Names
Ataxin-3; EC 3.4.19.12; Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein; ATXN3; ATX3; MJD; MJD1; SCA3
Product Gene Name
anti-ATX3 antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P54252
Species Reactivity
Human, Mouse, Rat
Purity/Purification
Affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen.
Form/Format
Liquid in PBS containing 50% glycerol, and 0.02% sodium azide.
Immunogen
Synthesized peptide derived from human protein. at AA range: 200-280
Preparation and Storage
Store at-20 degree C for 1 year.
Other Notes
Small volumes of anti-ATX3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Applications Tested/Suitable for anti-ATX3 antibody
Western Blot (WB), ELISA (EIA)
Application Notes for anti-ATX3 antibody
WB: 1:500-2000
ELISA: 1:5000-20000
NCBI/Uniprot data below describe general gene information for ATX3. It may not necessarily be applicable to this product.
NCBI Accession #
NP_001121168.1
[Other Products]
NCBI GenBank Nucleotide #
NM_001127696.1
[Other Products]
UniProt Primary Accession #
P54252
[Other Products]
UniProt Secondary Accession #
O15284; O15285; O15286; Q8N189; Q96TC3; Q96TC4; Q9H3N0; A7LFZ5; D6RDL9; E9PB63[Other Products]
UniProt Related Accession #
P54252[Other Products]
NCBI Official Full Name
ataxin-3 isoform ad
NCBI Official Synonym Full Names
ataxin 3
NCBI Official Symbol
ATXN3??[Similar Products]
NCBI Official Synonym Symbols
AT3; JOS; MJD; ATX3; MJD1; SCA3
??[Similar Products]
NCBI Protein Information
ataxin-3
UniProt Protein Name
Ataxin-3
UniProt Synonym Protein Names
Machado-Joseph disease protein 1; Spinocerebellar ataxia type 3 protein
Protein Family
Histone-lysine N-methyltransferase
UniProt Gene Name
ATXN3??[Similar Products]
UniProt Synonym Gene Names
ATX3; MJD; MJD1; SCA3??[Similar Products]
NCBI Summary for ATX3
Machado-Joseph disease, also known as spinocerebellar ataxia-3, is an autosomal dominant neurologic disorder. The protein encoded by this gene contains (CAG)n repeats in the coding region, and the expansion of these repeats from the normal 12-44 to 52-86 is one cause of Machado-Joseph disease. There is a negative correlation between the age of onset and CAG repeat numbers. Alternatively spliced transcript variants encoding different isoforms have been described for this gene. [provided by RefSeq, Jul 2016]
UniProt Comments for ATX3
Deubiquitinating enzyme involved in protein homeostasis maintenance, transcription, cytoskeleton regulation, myogenesis and degradation of misfolded chaperone substrates (PubMed:12297501, PubMed:17696782, PubMed:23625928, PubMed:28445460, PubMed:16118278). Binds long polyubiquitin chains and trims them, while it has weak or no activity against chains of 4 or less ubiquitins (PubMed:17696782). Involved in degradation of misfolded chaperone substrates via its interaction with STUB1/CHIP: recruited to monoubiquitinated STUB1/CHIP, and restricts the length of ubiquitin chain attached to STUB1/CHIP substrates and preventing further chain extension (). Interacts with key regulators of transcription and represses transcription: acts as a histone-binding protein that regulates transcription (PubMed:12297501). Regulates autophagy via the deubiquitination of 'Lys-402' of BECN1 leading to the stabilization of BECN1 (PubMed:28445460).
Research Articles on ATX3
1. Low exon skipping efficiencies combined with reduction in important ataxin-3 protein functions suggest that skipping of exon 8 and 9 is not a viable therapeutic option for spinocerebellar ataxia type-3. The modified protein was incapable of binding poly-ubiquitin chains, which may interfere with its normal deubiquitinating function.
Precautions
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Disclaimer
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