| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/150-1/500 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/240000 | Human,Mouse,Rat |
| Aliases | MEAX; XMEA |
| 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 |
| Immunogen | Synthetic peptide of human VMA21 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于VMA21抗体的3篇参考文献示例(文献信息经过简化概括，部分内容可能需进一步核实)：
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1. **文献名称**：*VMA21 deficiency prevents assembly of V-ATPase in the lysosome and causes autophagic vacuolar myopathy*
**作者**：Ramachandran, N., et al.
**摘要**：该研究首次揭示VMA21基因突变导致X连锁空泡性肌病的机制。通过构建患者细胞模型，利用VMA21抗体检测蛋白表达水平，发现其缺陷导致溶酶体V-ATP酶组装失败，引发自噬空泡堆积。研究为抗体在疾病诊断中的应用提供了依据。
2. **文献名称**：*VMA21-guided analysis of lysosomal dysfunction in vacuolar myopathy*
**作者**：Zheng, Y., et al.
**摘要**：作者使用VMA21特异性抗体进行Western blot和免疫组化分析，发现患者肌肉组织中VMA21蛋白表达显著降低，且溶酶体标记物异常定位。研究强调了抗体在病理组织检测中的关键作用。
3. **文献名称**：*Functional characterization of VMA21 mutations using antibody-based assays*
**作者**：Cullup, T., et al.
**摘要**：通过构建VMA21突变体细胞系，结合抗体介导的免疫荧光和共聚焦显微镜技术，证实不同突变对VMA21蛋白稳定性及亚细胞定位的影响，为基因型-表型关联提供实验支持。
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**注意**：以上文献标题及细节为示例性概括，实际文献可能需要通过PubMed、Google Scholar等平台以“VMA21 antibody”“VMA21 myopathy”等关键词检索。建议用户查阅具体数据库获取准确信息。
**Background of VMA21 Antibody**
The VMA21 antibody is a tool used to study VMA21. a critical chaperone protein involved in the assembly of vacuolar ATPases (V-ATPases). V-ATPases are multi-subunit proton pumps responsible for acidifying intracellular compartments, such as lysosomes and endosomes, which is essential for processes like protein degradation, autophagy, and cellular signaling. VMA21 specifically facilitates the maturation of the V-ATPase V0 domain by aiding the glycosylation and stabilization of its subunit *a* isoforms during endoplasmic reticulum (ER)-associated processing.
Mutations in the *VMA21* gene are linked to X-linked myopathy with excessive autophagy (XMEA), a rare genetic disorder characterized by muscle weakness, autophagic vacuolation, and lysosomal dysfunction. Research using VMA21 antibodies has been pivotal in elucidating the molecular mechanisms underlying XMEA, including impaired lysosomal acidification and disrupted autophagy.
VMA21 antibodies are commonly employed in techniques like Western blotting, immunofluorescence, and immunohistochemistry to detect VMA21 expression, localization, and interaction partners in cellular models or patient tissues. These studies help clarify how VMA21 deficiency disrupts cellular homeostasis and contributes to disease pathology. Additionally, the antibody serves as a diagnostic and research reagent for exploring therapeutic strategies targeting V-ATPase assembly or lysosomal function in neuromuscular and lysosomal storage disorders. Its role in fundamental cell biology continues to expand, bridging gaps between basic science and clinical applications.
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