| WB | 1/500-1/8000 | 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 | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Torsin-1A, Dystonia 1 protein, Torsin ATPase-1A, 364-, Torsin family 1 member A, TOR1A, DQ2, DYT1, TA, TORA |
| Entrez GeneID | 1861 |
| WB Predicted band size | 37.9kDa |
| 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 |
| Immunogen | Purified His-tagged DYT1 protein(Fragment) was used to produced this monoclonal antibody. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于DYT1相关抗体的模拟参考文献示例(基于研究背景构建，非真实存在的文献)：
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1. **文献名称**: "Monoclonal Antibody Development for TOR1A Protein in DYT1 Dystonia"
**作者**: Ozelius LJ, et al.
**摘要**: 本研究报道了一种特异性识别TOR1A蛋白(DYT1肌张力障碍致病蛋白)的单克隆抗体的开发。通过Western blot和免疫组化验证，该抗体能够灵敏检测人脑组织及细胞模型中的TOR1A表达，为研究突变体蛋白(如ΔE302/303缺失突变)的异常定位提供了工具。
2. **文献名称**: "Characterization of DYT1 Antibody for Functional Studies in Neuronal Models"
**作者**: Bragg DC, Breakefield XO
**摘要**: 文章描述了一种多克隆抗体的应用，该抗体靶向TOR1A蛋白的C端结构域。实验显示，该抗体可用于研究DYT1突变对神经元内质网应激反应的影响，并揭示突变蛋白与分子伴侣的异常相互作用。
3. **文献名称**: "Immunohistochemical Analysis of TorsinA in Postmortem Dystonia Brains"
**作者**: Hettich J, et al.
**摘要**: 利用商业化的DYT1抗体(克隆号TA-2A5)，本研究对比了健康人与DYT1肌张力障碍患者脑组织中TorsinA的分布差异，发现突变蛋白在纹状体和丘脑中的聚集现象，提示其与运动通路功能障碍的潜在关联。
4. **文献名称**: "Antibody-Based Detection of TorsinA Oligomers in Cellular Stress Models"
**作者**: Gómez-Herreros F, et al.
**摘要**: 通过开发一种新型抗体，本研究证实了氧化应激条件下TOR1A蛋白可形成寡聚体，且DYT1突变体(ΔE)的寡聚倾向显著增强，为解析疾病分子机制提供了实验依据。
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**注**：以上文献为示例性内容，实际研究中请通过PubMed或Google Scholar检索真实文献(关键词如“DYT1 antibody”、“TOR1A antibody”、“TorsinA detection”)。真实研究可能涉及以下学者：Laurie Ozelius、Xandra Breakefield、William Dauer等。
The DYT1 antibody is primarily associated with research on DYT1 dystonia, a hereditary movement disorder caused by mutations in the *TOR1A* gene. This gene encodes torsinA, a protein belonging to the AAA+ ATPase family, which plays a role in cellular processes like membrane trafficking, cytoskeletal dynamics, and endoplasmic reticulum stress response. The most common mutation, a GAG deletion (ΔE302/303), leads to a truncated torsinA protein, disrupting its normal function and contributing to neuronal dysfunction in basal ganglia and cerebellar circuits.
DYT1 antibodies are tools developed to detect torsinA expression and localization in cellular and animal models. They help researchers investigate how mutant torsinA disrupts nuclear membrane integrity, causes protein aggregation, or impairs synaptic vesicle recycling. Such studies aim to unravel the pathophysiology of DYT1 dystonia, characterized by involuntary muscle contractions, repetitive movements, or abnormal postures. Antibodies are also used in immunohistochemistry, Western blotting, and immunofluorescence to compare torsinA distribution in wild-type versus mutant systems, aiding in biomarker discovery or therapeutic target validation.
While DYT1 antibodies are not used diagnostically (genetic testing confirms *TOR1A* mutations), they remain vital in preclinical research, including drug screening or gene therapy approaches aimed at mitigating torsinA dysfunction. Their application has advanced understanding of how torsinA interacts with cofactors like LAP1 or LULL1. offering insights into broader mechanisms underlying dystonia and related neurological disorders.
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