| WB | 1/500 - 1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 1/200 - 1/400 | Human,Mouse,Rat |
| Elisa | 1/10000 | Human,Mouse,Rat |
| Aliases | ATX1; SCA1; D6S504E |
| Entrez GeneID | 6310 |
| clone | 2B8A2 |
| WB Predicted band size | 86.9kDa |
| Host/Isotype | Mouse IgG1 |
| 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 | Human,Mouse |
| Immunogen | Purified recombinant fragment of human ATXN1 (AA: 645-815) expressed in E. Coli. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide |
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以下是关于ATXN1抗体的3篇代表性文献的虚构示例(基于领域知识推测，实际文献需通过数据库验证)：
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1. **文献名称**：*"Immunodetection of mutant ATXN1 aggregates in spinocerebellar ataxia type 1"*
**作者**：Orr HT, Zoghbi HY
**摘要**：研究通过特异性ATXN1抗体，在小鼠模型和患者组织中检测突变ATXN1蛋白的核内聚集，揭示其与神经元损伤的相关性，为SCA1病理机制提供证据。
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2. **文献名称**：*"Development of a phospho-specific ATXN1 antibody for studying SCA1 pathogenesis"*
**作者**：Duvick L, et al.
**摘要**：开发了一种针对ATXN1磷酸化位点的抗体，证实磷酸化修饰影响ATXN1的稳定性及毒性，为靶向治疗提供了分子标记。
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3. **文献名称**：*"Comparative analysis of commercial ATXN1 antibodies in neurodegenerative disease models"*
**作者**：Serra HG, Al-Ramahi I
**摘要**：评估多种商用ATXN1抗体的特异性和灵敏度，筛选出适用于免疫组化、Western blot的可靠抗体，优化实验条件。
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**提示**：建议通过PubMed或Google Scholar检索真实文献，关键词如“ATXN1 antibody”、“ataxin-1 immunodetection”或结合特定应用场景(如“SCA1 antibody validation”)。知名研究团队(如Zoghbi实验室)的论文可能更权威。
ATXN1 antibodies are essential tools in studying the molecular mechanisms of spinocerebellar ataxia type 1 (SCA1), a neurodegenerative disorder caused by mutations in the *ATXN1* gene. The *ATXN1* gene, located on chromosome 6. encodes the ataxin-1 protein, which contains a polyglutamine (polyQ) tract. In SCA1. an abnormal expansion of CAG repeats (≥39 repeats) within this tract leads to a toxic, misfolded ataxin-1 protein that aggregates in neuronal nuclei, disrupting cellular functions and causing cerebellar degeneration. ATXN1 antibodies are designed to detect either wild-type or mutant forms of the protein, aiding in research on disease pathogenesis, protein localization, and aggregation dynamics.
These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to study ATXN1 expression in cell lines, animal models (e.g., *Atxn1* knockout or transgenic mice), and patient-derived samples. Specific clones may target distinct epitopes, such as the N-terminal region or polyQ domain, enabling differentiation between normal and pathogenic variants. Additionally, ATXN1 antibodies help investigate post-translational modifications (e.g., phosphorylation at Ser776) critical for protein stability and toxicity.
Research using ATXN1 antibodies has also explored therapeutic strategies, including antisense oligonucleotides (ASOs) or small molecules to reduce mutant protein levels. Understanding ATXN1’s interactions with RNA-binding proteins and its role in transcriptional regulation further highlights its significance in neuronal homeostasis. Overall, ATXN1 antibodies remain pivotal in unraveling SCA1 pathology and advancing targeted therapies.
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