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Rabbit Anti-ATXN1 Antibody (CSB-PA002438ESR2HU)
Supplier: CUSABIO BIOTECH CO.
Spinocerebellar ataxia type 1 protein Antibody,ATXN1 Antibody,ATX1 Antibody, SCA1 AntibodyMore alternative names for the antibody
alternative ataxin1 antibody|Ataxin-1 antibody|ATX1 antibody|ATX1_HUMAN antibody|Atxn1 antibody|D6S504E antibody|OTTHUMP00000016065 antibody|SCA1 antibody|Spinocerebellar ataxia type 1 protein antibody
Anti-Ataxin 1 antibody [S768] (ab186265)
Anti-Ataxin 1 antibody [S768] (ab186265)
Recommended applications: ELISA, IHC
Recommended dilution: Recommended dilution:IHC:1:20-1:200
Recommended protocols: check protocols
|Catalogue No.|| |
Recombinant human Ataxin-1 protein (586-815AA)
|Recommended dilution|| |
|Molecular weight|| |
Antigen Affinity Purified
Shipped at 4 Celcius Degree. Upon delivery aliquot and store at -20 Celcius Degree or -80 Celcius Degree. Avoid repeated freeze.
|Database links|| |
<a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=6310" target="_blank">Entrez Gene:6310( Human)</a>, <a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=20238" target="_blank">Entrez Gene:20238( Mouse)</a>, <a href="http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=25049" target="_blank">Entrez Gene:25049( Rat)</a>, <a href="http://www.ncbi.nlm.nih.gov/omim/601556" target="_blank">Omim:601556( Human)</a>, <a href="http://www.uniprot.org/uniprot/P54253" target="_blank">SwissProt:P54253( Human)</a>, <a href="http://www.uniprot.org/uniprot/P54254" target="_blank">SwissProt:P54254( Mouse)</a>, <a href="http://www.uniprot.org/uniprot/Q63540" target="_blank">SwissProt:Q63540( Rat)</a>, <a href="http://www.ncbi.nlm.nih.gov/UniGene/clust.cgi?ORG=Hs&CID=434961" target="_blank">Unigene:434961( Human)</a>, <a href="http://www.ncbi.nlm.nih.gov/UniGene/clust.cgi?ORG=Mm&CID=342683" target="_blank">Unigene:342683( Mouse)</a>, <a href="http://www.ncbi.nlm.nih.gov/UniGene/clust.cgi?ORG=Mm&CID=342686" target="_blank">Unigene:342686( Mouse)</a>, <a href="http://www.ncbi.nlm.nih.gov/UniGene/clust.cgi?ORG=Rn&CID=88438" target="_blank">Unigene:88438( Rat)</a>
|Protein function|| |
Chromatin-binding factor that repress Notch signaling in the absence of Notch intracellular domain by acting as a CBF1 corepressor. Binds to the HEY promoter and might assist, along with NCOR2, RBPJ-mediated repression. Binds RNA in vitro. May be involved in RNA metabolism. .
|Protein tissue specificity|| |
Widely expressed throughout the body. .
|Involvement in disease|| |
Spinocerebellar ataxia 1 (SCA1) [MIM:164400]: Spinocerebellar ataxia is a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to cerebellum degeneration with variable involvement of the brainstem and spinal cord. SCA1 belongs to the autosomal dominant cerebellar ataxias type I (ADCA I) which are characterized by cerebellar ataxia in combination with additional clinical features like optic atrophy, ophthalmoplegia, bulbar and extrapyramidal signs, peripheral neuropathy and dementia. SCA1 is caused by expansion of a CAG repeat in the coding region of ATXN1. Longer expansions result in earlier onset and more severe clinical manifestations of the disease. . Note=The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by expansion of the polyglutamine tract to about 40-83 repeats, causing accumulation in neurons and exerting toxicity. .
|Protein sequence and domain|| |
Belongs to the ATXN1 family. The AXH domain is required for interaction with CIC.
|Protein post-translational modifications|| |
Ubiquitinated by UBE3A, leading to its degradation by the proteasome. The presence of expanded poly-Gln repeats in spinocerebellar ataxia 1 (SCA1) patients impairs ubiquitination and degradation, leading to accumulation of ATXN1 in neurons and subsequent toxicity. .; Phosphorylation at Ser-775 increases the pathogenicity of proteins with an expanded polyglutamine tract. .; Sumoylation is dependent on nuclear localization and phosphorylation at Ser-775. It is reduced in the presence of an expanded polyglutamine tract. .
|Protein cellular localization|| |
Cytoplasm . Nucleus . Note=Colocalizes with USP7 in the nucleus. .
|Research area|| |
<a href="https://antibodyplus.com/research-areas/">All research areas</a>><a href="https://antibodyplus.com/Signaling-Intermediates/">Signaling Intermediates</a>><a href="https://antibodyplus.com/product-tag/Ataxin/">Ataxin</a><br><a href="https://antibodyplus.com/product-tag/Signaling-Intermediates/"> (View all antibody categories related to Signaling Intermediates)</a>
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|Product type|| |
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