| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/25-1/100 | 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 | SAP-3; GM2-AP |
| WB Predicted band size | 21 kDa |
| 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 | Fusion protein of human GM2A |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是关于GM2A抗体的3篇代表性文献的简要信息：
---
1. **文献名称**：*"The role of GM2 activator protein in Tay-Sachs disease pathogenesis"*
**作者**：Chen H, et al.
**摘要**：研究通过免疫印迹和免疫组化技术，利用GM2A特异性抗体分析其在泰-萨克斯病模型中的表达变化，发现GM2A蛋白缺失导致GM2神经节苷脂在溶酶体中异常积累，加剧神经元退行性变。
2. **文献名称**：*"Autoantibodies against GM2 activator protein in multiple sclerosis patients"*
**作者**：Smith JL, et al.
**摘要**：首次报道在多发性硬化症患者血清中检测到抗GM2A的自身抗体，提示其可能通过干扰鞘脂代谢参与脱髓鞘病理过程，为自身免疫机制提供了新视角。
3. **文献名称**：*"Development of a monoclonal antibody specific for human GM2 activator protein"*
**作者**：Lee S, et al.
**摘要**：描述了一种高亲和力抗人GM2A单克隆抗体的制备与验证，该抗体能精准识别病理组织中的GM2A蛋白，为溶酶体贮积症的诊断提供了新型工具。
---
注：上述文献为示例性质，实际研究中建议通过PubMed或Web of Science等平台以“GM2A antibody”“GM2 activator protein”等关键词检索最新文献。
GM2A antibody targets the GM2 activator protein, a crucial cofactor in the lysosomal degradation of GM2 gangliosides. GM2 gangliosides are sphingolipids abundant in neuronal cell membranes, and their metabolism relies on the GM2 activator protein (GM2AP) to solubilize GM2 and present it to β-hexosaminidase A for cleavage. Dysregulation of this process, due to genetic mutations in GM2AP, underlies the rare lysosomal storage disorder AB-variant GM2 gangliosidosis.
Anti-GM2A antibodies are primarily associated with autoimmune neuropathy, particularly Guillain-Barré syndrome (GBS) and its variants. These autoantibodies may interfere with GM2 ganglioside metabolism or directly target neuronal structures, contributing to immune-mediated nerve damage. While anti-GM1 or anti-GQ1b antibodies are more commonly linked to specific GBS subtypes (e.g., acute motor axonal neuropathy or Miller Fisher syndrome), GM2A antibodies are less frequently detected and their clinical significance remains under investigation.
Detection typically involves ELISA or immunoblotting using purified GM2AP. Research suggests these antibodies may correlate with specific neurological symptoms, though their role as biomarkers or pathogenic drivers requires further validation. Current studies focus on clarifying their diagnostic utility and exploring potential cross-reactivity with structurally related gangliosides in autoimmune neuropathy.
×
