| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | ATX1; SCA1; D6S504E |
| Entrez GeneID | 6310 |
| WB Predicted band size | Calculated MW: 87 kDa; Observed MW: 105 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Mouse |
| Immunogen | Recombinant protein of human Ataxin 1 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
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以下是关于Ataxin 1抗体的3篇参考文献及其摘要概括：
1. **文献名称**：*"Expression analysis of ataxin-1 mRNA and protein in human brain: Evidence for a widespread distribution and focal protein accumulation"*
**作者**：Servadio, A., et al.
**摘要**：该研究通过Northern blot和免疫组化技术，利用Ataxin 1特异性抗体揭示了其在人脑中的广泛表达，并发现突变型Ataxin 1在SCA1患者小脑神经元中形成核内包涵体，提示蛋白异常聚集与疾病相关。
2. **文献名称**：*"Allele-specific silencing of mutant ataxin-1 by small interfering RNA molecules"*
**作者**：Xia, H., et al.
**摘要**：研究开发了靶向突变型Ataxin 1的siRNA，并利用Ataxin 1抗体验证其特异性敲低效果，结果显示突变蛋白水平显著下降，为SCA1的基因治疗提供了实验依据。
3. **文献名称**：*"Phosphorylation regulates proteasomal-mediated degradation and solubility of ataxin-1"*
**作者**：Duvick, L., et al.
**摘要**：通过磷酸化特异性Ataxin 1抗体，研究发现Ser776位点的磷酸化修饰增强了突变蛋白的稳定性和毒性，抗体检测揭示了其在蛋白降解途径中的关键作用。
4. **文献名称**：*"Identification of a self-association region within the SCA1 gene product, ataxin-1"*
**作者**：Burright, E.N., et al.
**摘要**：该研究利用抗Ataxin 1抗体进行免疫共沉淀实验，发现其AXH结构域介导自身寡聚化，为突变蛋白异常聚集机制提供了分子基础。
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以上文献均聚焦于Ataxin 1抗体的应用，涵盖蛋白定位、修饰分析和功能研究，适用于疾病机制探索及治疗开发。
Ataxin-1 is a protein encoded by the *ATXN1* gene, primarily associated with spinocerebellar ataxia type 1 (SCA1), an autosomal dominant neurodegenerative disorder. In SCA1. a CAG trinucleotide repeat expansion in *ATXN1* leads to an elongated polyglutamine tract in the Ataxin-1 protein, resulting in its misfolding, aggregation, and accumulation in neuronal nuclei. This triggers neurotoxicity, particularly in cerebellar Purkinje cells and brainstem neurons, leading to progressive motor dysfunction.
Antibodies targeting Ataxin-1 are critical tools for studying its role in SCA1 pathogenesis. They enable detection of wild-type and mutant protein expression, localization, and aggregation in cellular and animal models. Commonly used in techniques like Western blotting, immunohistochemistry (IHC), and immunofluorescence (IF), these antibodies help characterize Ataxin-1’s normal functions (e.g., RNA processing, transcriptional regulation) and its pathological behavior in disease. Specific epitopes, such as the polyglutamine region or distinct domains, allow differentiation between normal and expanded forms.
Research utilizing Ataxin-1 antibodies has advanced understanding of SCA1 mechanisms, including interactions with protein partners (e.g., CIC, RBM17) and modifiers of toxicity. They also support therapeutic development, such as evaluating gene silencing or aggregation inhibitors. Validation of antibody specificity remains essential, given potential cross-reactivity with homologous proteins. Overall, Ataxin-1 antibodies are indispensable for unraveling molecular pathways in SCA1 and identifying potential interventions.
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