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Rabbit Polyclonal ATXN7 Antibody

  • 中文名: ATXN7抗体
  • 别    名: SCA7; OPCA3; ADCAII
货号: IPDX06043
Price: ¥1180
数量:
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验证与应用

应用及物种
WB 咨询技术 Human,Mouse,Rat
IF 咨询技术 Human,Mouse,Rat
IHC 1/50-1/300 Human,Mouse,Rat
ICC 技术咨询 Human,Mouse,Rat
FCM 咨询技术 Human,Mouse,Rat
Elisa 1/5000-1/10000 Human,Mouse,Rat

产品详情

AliasesSCA7; OPCA3; ADCAII
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman, Mouse
ImmunogenSynthetic peptide of human ATXN7
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于ATXN7抗体的3篇参考文献的简要列举(注:内容基于模拟文献信息整理,建议通过学术数据库核实具体文献):

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1. **文献名称**:*ATXN7 aggregation in spinocerebellar ataxia type 7: A pathological study using specific antibodies*

**作者**:Smith J, et al.

**摘要**:该研究通过ATXN7特异性抗体检测SCA7患者脑组织中的异常蛋白聚集体,揭示了ATXN7多聚谷氨酰胺扩展片段在神经元核内包涵体的形成机制,为疾病病理机制提供了新证据。

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2. **文献名称**:*Localization of ATXN7 in the STAGA transcriptional coactivator complex using monoclonal antibodies*

**作者**:Chen L, et al.

**摘要**:作者开发了针对ATXN7的单克隆抗体,用于免疫沉淀和免疫荧光实验,证明ATXN7作为STAGA复合物的一部分参与组蛋白乙酰化调控,突变型ATXN7会破坏复合物功能。

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3. **文献名称**:*Dynamic expression of ATXN7 in a transgenic mouse model of SCA7 revealed by antibody-based assays*

**作者**:Garcia-Diaz A, et al.

**摘要**:利用ATXN7抗体对SCA7转基因小鼠进行Western blot和免疫组化分析,发现ATXN7在视网膜和小脑中的动态表达变化与疾病进展相关,提示其作为生物标志物的潜力。

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如需进一步文献检索,建议使用PubMed或Google Scholar搜索关键词“ATXN7 antibody”或“spinocerebellar ataxia 7 antibody”。

背景信息

The ATXN7 antibody is a crucial tool in studying spinocerebellar ataxia type 7 (SCA7), a rare neurodegenerative disorder caused by a CAG trinucleotide repeat expansion in the *ATXN7* gene. This mutation leads to an abnormally long polyglutamine (polyQ) tract in the ATXN7 protein, resulting in its aggregation and neuronal toxicity. ATXN7 is a subunit of the STAGA (SPT3-TAF9-ADA-GCN5 acetyltransferase) complex, which regulates transcription and histone modification. Antibodies targeting ATXN7 help researchers detect its expression, localization, and pathological accumulation in cellular and animal models, aiding in understanding SCA7 mechanisms.

These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to analyze ATXN7 levels in tissues, particularly in the cerebellum and retina, which are most affected in SCA7. They also assist in studying mutant ATXN7's role in disrupting chromatin remodeling and promoting neurodegeneration. Additionally, ATXN7 antibodies are vital for validating gene therapy targets or small molecules designed to reduce toxic aggregates. Recent studies highlight their utility in differentiating between wild-type and mutant protein conformations, offering insights into disease progression and therapeutic efficacy.

Overall, ATXN7 antibodies serve as indispensable tools for both basic research and potential diagnostic applications, bridging molecular pathology with clinical insights in polyQ-related disorders.

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