| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20-1/50 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/20-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Alpha gal A; GALA; Galactosidase, alpha; GLA; Melibiase;;alpha Galactosidase A |
| WB Predicted band size | Calculated MW: 49 kDa ; Observed MW: 46 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 |
| Immunogen | A synthesized peptide derived from human alpha Galactosidase A |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是3-4条关于 **Galactosidase alpha(GLA，α-半乳糖苷酶)抗体** 的参考文献示例(内容基于公开文献概括)：
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1. **"Antibody-Mediated Inhibition of α-Galactosidase A in Fabry Disease"**
- **作者**: Linthorst, G.E., et al.
- **摘要**: 研究法布里病患者中抗α-半乳糖苷酶A抗体的产生及其对酶替代疗法(ERT)的影响，发现部分患者体内抗体会中和外源性GLA活性，降低治疗效果。
2. **"Development of a Novel Monoclonal Antibody for Specific Detection of α-Galactosidase A in Human Tissues"**
- **作者**: Sakuraba, H., et al.
- **摘要**: 报道一种高特异性单克隆抗体的开发，用于免疫组化检测人类组织中α-半乳糖苷酶A的表达水平，为法布里病病理机制研究提供工具。
3. **"Impact of Anti-Drug Antibodies on Clinical Outcomes in Fabry Disease During Enzyme Replacement Therapy"**
- **作者**: Germain, D.P., et al.
- **摘要**: 分析长期接受ERT的法布里病患者中抗GLA抗体的发生率及其与临床指标(如肾功能和疼痛评分)的关联，提示抗体可能影响部分患者预后。
4. **"Immunological Characterization of α-Galactosidase A Mutants for Diagnosis of Fabry Disease"**
- **作者**: Arends, M., Biegstraaten, M.
- **摘要**: 利用抗GLA抗体结合质谱技术，鉴别法布里病相关GLA基因突变导致的酶功能异常，为精准诊断提供新方法。
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**注**：以上为示例性内容，具体文献需通过学术数据库(如PubMed、Web of Science)以关键词“α-galactosidase antibody”“Fabry disease GLA”检索获取最新研究。
Galactosidase alpha (α-galactosidase A, GLA) antibodies are immunological tools specifically designed to detect and study the α-galactosidase A enzyme, a lysosomal hydrolase critical for glycolipid metabolism. This enzyme catalyzes the hydrolysis of globotriaosylceramide (Gb3) and other glycosphingolipids, and its deficiency leads to Fabry disease, a rare X-linked lysosomal storage disorder. Antibodies against GLA are widely used in research and diagnostics to assess enzyme expression, localization, and activity in tissues or cell cultures, aiding in the understanding of Fabry disease pathogenesis.
These antibodies are employed in techniques like ELISA, Western blotting, and immunohistochemistry to quantify protein levels, evaluate mutations, or monitor therapeutic efficacy in enzyme replacement therapy (ERT) or gene therapy. Commercially available monoclonal or polyclonal GLA antibodies target specific epitopes, enabling precise detection of both endogenous and recombinant forms of the enzyme. Additionally, they help identify cross-reactive immunological material (CRIM) in patients, guiding personalized treatment strategies. In clinical diagnostics, GLA antibodies assist in confirming Fabry disease by detecting reduced enzyme activity or abnormal protein accumulation. Their role extends to developing novel therapies, including substrate reduction therapy and chaperone-mediated approaches. As research advances, GLA antibodies remain pivotal in bridging molecular insights with therapeutic innovations for lysosomal disorders.
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