| Host: | Rabbit |
| Applications: | WB/IP |
| Reactivity: | Mouse |
| Note: | STRICTLY FOR FURTHER SCIENTIFIC RESEARCH USE ONLY (RUO). MUST NOT TO BE USED IN DIAGNOSTIC OR THERAPEUTIC APPLICATIONS. |
| Short Description: | Rabbit monoclonal antibody anti-Ataxin 1 (562-693 aa) is suitable for use in Western Blot and Immunoprecipitation research applications. |
| Clonality: | Monoclonal |
| Clone ID: | R01-8H1 |
| Conjugation: | Unconjugated |
| Isotype: | IgG |
| Formulation: | 50mM Tris-Glycine (pH7.4) , 0.15M NaCl, 40% Glycerol, 0.01% Sodium azide and 0.05% BSA |
| Purification: | Affinity Purified |
| Concentration: | 0.3 mg/ml |
| Dilution Range: | WB 1:500-1:1000 IP 1:20 |
| Storage Instruction: | Store at 4°C short term. Aliquot and store at-20°C long term. Avoid freeze/thaw cycles. |
| Gene Symbol: | ATXN1 |
| Gene ID: | 6310 |
| Uniprot ID: | ATX1_HUMAN |
| Immunogen Region: | 562-693 aa |
| Immunogen: | Recombinant protein of human Ataxin 1 |
| Tissue Specificity | Widely expressed throughout the body. |
| 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. |
| 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. In concert with CIC and ATXN1L, involved in brain development. |
| Protein Name | Ataxin-1 Spinocerebellar Ataxia Type 1 Protein |
| Cellular Localisation | Cytoplasm Nucleus Colocalizes With Usp7 In The Nucleus |
| Alternative Antibody Names | Anti-Ataxin-1 antibody Anti-Spinocerebellar Ataxia Type 1 Protein antibody Anti-ATXN1 antibody Anti-ATX1 antibody Anti-SCA1 antibody |
Information sourced from Uniprot.org
