| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/40-1/200 | 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 |
| Aliases | SYM1; MTDPS6 |
| 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 | Human, Mouse, Rat |
| Immunogen | Fusion protein of human MPV17 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3篇与MPV17抗体相关的研究文献示例(文献信息为模拟概括，仅供参考)：
1. **文献名称**: "MPV17 mutations associated with mitochondrial DNA depletion syndrome: Immunohistochemical analysis of liver tissue using a novel anti-MPV17 antibody"
**作者**: Johnson R, et al.
**摘要**: 本研究开发了一种针对人MPV17蛋白的单克隆抗体，并验证其在肝组织中的特异性。通过免疫组化技术分析MPV17突变患者的肝脏样本，发现MPV17蛋白表达显著降低，且与线粒体DNA拷贝数减少相关，为临床诊断提供了生物标志物检测工具。
2. **文献名称**: "Functional characterization of MPV17 in mitochondrial inner membrane integrity using antibody-mediated protein localization"
**作者**: Chen L, et al.
**摘要**: 利用商业化MPV17抗体进行免疫荧光和Western blot实验，揭示了MPV17蛋白在线粒体内膜中的定位模式。通过敲低细胞模型发现，MPV17缺失导致线粒体膜电位异常和活性氧(ROS)累积，提示其在维持线粒体膜稳定性中的关键作用。
3. **文献名称**: "Development of a polyclonal antibody against the N-terminal domain of MPV17 and its application in zebrafish disease modeling"
**作者**: Müller S, et al.
**摘要**: 研究团队制备了针对MPV17 N端结构域的多克隆抗体，并在斑马鱼模型中验证其交叉反应性。通过抗体检测发现，MPV17缺陷型斑马鱼表现出与人类肝脑型线粒体病相似的表型，为疾病机制研究提供了新模型。
4. **文献名称**: "Comparative analysis of MPV17 antibody specificity across mammalian species for translational research"
**作者**: Gupta A, et al.
**摘要**: 系统性评估了多种市售MPV17抗体在小鼠、大鼠及人类细胞中的特异性差异，发现部分抗体存在种属交叉反应性不足的问题。研究为跨物种实验中的抗体选择提供了重要参考依据，强调了验证抗体适用性的必要性。
注：以上文献为知识库构建示例，实际研究需通过PubMed/Google Scholar等平台检索真实论文。建议结合关键词"MPV17 antibody validation"或"MPV17 immunohistochemistry"查找最新文献。
The MPV17 antibody is a crucial tool in studying the MPV17 protein, a mitochondrial inner membrane protein encoded by the *MPV17* gene. This protein plays a vital role in maintaining mitochondrial DNA (mtDNA) integrity and regulating oxidative phosphorylation. Mutations in *MPV17* are linked to mitochondrial DNA depletion syndromes (MDDS), particularly hepatocerebral forms characterized by liver dysfunction and neurological impairments. Researchers use MPV17 antibodies to investigate the protein's expression, localization, and functional interactions within mitochondria, aiding in the diagnosis and mechanistic understanding of related disorders.
MPV17 antibodies are widely employed in techniques like Western blotting, immunohistochemistry, and immunofluorescence to detect protein levels in tissues or cell lines. Studies also explore MPV17's role beyond genetic diseases, including its potential involvement in mitochondrial dysfunction associated with aging, neurodegenerative diseases, and metabolic disorders. Additionally, research suggests MPV17 may influence cellular responses to oxidative stress and apoptosis, linking it to broader pathophysiological processes. The antibody's specificity and reliability are critical for validating experimental models, such as *MPV17*-knockout mice, which recapitulate human disease phenotypes. By enabling precise tracking of MPV17 expression changes under various conditions, these antibodies contribute to advancing therapeutic strategies, including gene therapy or small-molecule interventions targeting mitochondrial pathways.
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