| WB | 1/4000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | 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 | N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH, HSS |
| Entrez GeneID | 6448 |
| WB Predicted band size | 56.7kDa |
| 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 | This SGSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 420-449 amino acids from the C-terminal region of human SGSH. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于SGSH抗体的3篇参考文献示例(注：文献信息为模拟，实际引用需核实)：
1. **标题**：*AAV-mediated gene therapy restores sulfamidase activity in MPS IIIA mice*
**作者**：Bigger BW, et al.
**摘要**：研究通过腺相关病毒(AAV)递送SGSH基因至MPS IIIA模型小鼠大脑，利用SGSH抗体通过免疫组化和Western blot证实了酶表达的恢复，并观察到硫酸乙酰肝素积累减少及神经行为改善。
2. **标题**：*Characterization of sulfamidase-deficient iPSCs for modeling MPS IIIA*
**作者**：Fraldi A, et al.
**摘要**：通过患者来源的诱导多能干细胞(iPSCs)分化为神经细胞，使用SGSH抗体检测细胞内酶的表达缺失，并结合基因编辑技术修复突变后观察到酶活性恢复，为疾病机制研究提供模型。
3. **标题**：*Targeting sulfamidase in lysosomal storage disorders: Antibody validation and therapeutic potential*
**作者**：García-Filion H, et al.
**摘要**：验证了SGSH抗体的特异性，应用于检测不同物种(小鼠、非人灵长类)组织中的酶分布，并评估其在基因治疗和酶替代疗法中的药效学监测作用。
如需具体文献，建议通过PubMed或Google Scholar以关键词“SGSH antibody”、“sulfamidase antibody”或“MPS IIIA antibody”检索近年研究。
SGSH antibodies are immunological tools specifically designed to target sulfamidase, an enzyme encoded by the *SGSH* gene. This lysosomal enzyme, sulfamidase (N-sulfoglucosamine sulfohydrolase), plays a critical role in breaking down heparan sulfate, a complex glycosaminoglycan (GAG). Mutations in *SGSH* lead to impaired heparan sulfate degradation, causing its accumulation in lysosomes. This results in mucopolysaccharidosis type IIIA (MPS IIIA or Sanfilippo syndrome type A), a severe neurodegenerative lysosomal storage disorder characterized by developmental regression, behavioral issues, and early mortality.
SGSH antibodies are primarily used in research and diagnostics to study sulfamidase expression, localization, and function. They enable detection of the enzyme via techniques like Western blotting, immunohistochemistry, or ELISA, aiding in the identification of protein deficiencies in patient samples. Additionally, these antibodies support therapeutic development, such as monitoring enzyme replacement therapies (ERT) or gene therapy efficacy in preclinical models. Some antibodies differentiate between precursor and mature sulfamidase forms, providing insights into post-translational processing defects.
As MPS IIIA lacks a cure, SGSH antibodies remain vital for understanding disease mechanisms and advancing targeted treatments. Their specificity ensures accurate assessment of sulfamidase levels, crucial for both basic research and translational applications aimed at mitigating this devastating disorder.
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