| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | 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 | Alpha gal A; GALA; Galactosidase; alpha; GLA; Melibiase |
| Entrez GeneID | 2717 |
| WB Predicted band size | Calculated MW: 49 kDa; Observed MW: 49 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 synthetic peptide of human Galactosidase alpha |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
+ +
以下是关于α-Galactosidase抗体的3篇参考文献，包含文献名称、作者及摘要概述：
---
1. **文献名称**: "Antibody-Mediated Inhibition of α-Galactosidase A in Fabry Disease"
**作者**: Linthorst GE, Hollak CE, et al.
**摘要**: 研究探讨了法布里病患者接受酶替代疗法(ERT)后产生的抗体对α-galactosidase A酶活性的中和效应。发现部分患者体内抗体会降低酶疗效，影响长期治疗效果。
---
2. **文献名称**: "Structural Analysis of Human α-Galactosidase A for Antibody Epitope Mapping"
**作者**: Garman SC, Garboczi DN.
**摘要**: 通过X射线晶体学解析人源α-galactosidase A的三维结构，揭示了其抗原表位，为开发特异性抗体及研究抗体-酶相互作用机制提供了结构基础。
---
3. **文献名称**: "Development of Monoclonal Antibodies Against α-Galactosidase for Diagnostic Applications"
**作者**: Kobayashi T, Shimada Y, et al.
**摘要**: 报道了针对α-galactosidase的单克隆抗体制备及特性分析，验证了其在Western blot和免疫组化中的高特异性，适用于法布里病的早期诊断和酶活性检测。
---
4. **文献名称**: "Neutralizing Antibodies in Fabry Patients Receiving Agalsidase Alfa: Impact on Clinical Outcomes"
**作者**: Schiffmann R, Warnock DG, et al.
**摘要**: 长期追踪法布里病患者的抗体产生情况，发现部分患者的中和抗体会降低酶替代疗法的有效性，提示需个体化调整治疗方案。
---
这些文献覆盖了抗体在诊断、结构研究及治疗中的关键作用，可根据实际需求进一步扩展。
Alpha-galactosidase (α-Gal A), encoded by the *GLA* gene, is a lysosomal enzyme that catalyzes the hydrolysis of glycosphingolipids, particularly globotriaosylceramide (Gb3). Deficiency in α-Gal A activity causes Fabry disease, an X-linked lysosomal storage disorder characterized by Gb3 accumulation in multiple organs, leading to progressive renal, cardiac, and cerebrovascular complications. Antibodies targeting α-Gal A are critical tools in research, diagnostics, and therapeutic development.
These antibodies are widely used to detect α-Gal A expression levels in tissues or cells via techniques like Western blot, immunohistochemistry, or ELISA, aiding in Fabry disease diagnosis and mechanistic studies. They also play roles in monitoring recombinant enzyme replacement therapies (ERTs), such as agalsidase alfa, by assessing enzyme uptake or stability. Additionally, some antibodies are engineered to modulate enzyme activity or target α-Gal A for degradation studies.
Monoclonal and polyclonal α-Gal A antibodies are commercially available, often validated for specificity across species. Recent research explores their use in gene therapy monitoring or as companion diagnostics for emerging therapies. Understanding α-Gal A's structure-function relationships through antibody-based studies continues to inform therapeutic strategies for Fabry disease and related lysosomal disorders.
×
