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- Bioss
- bs-7593P
- 2025年10月16日
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- 详细信息
- 技术资料
- 规格：
500ug
| 产品编号 | bs-7593P |
| 英文名称 | GLA Antibody Blocking Peptide |
| 中文名称 | α-半乳糖苷酶封闭多肽 |
| 英文别名 | Galactosidase alpha; Alpha D galactosidase A; Alpha D galactoside galactohydrolase; Melibiase; Alpha galactosidase A; GALA; GLA; GLA protein; AGAL_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Cardiovascular > Blood Cardiovascular > Blood > Blood Cell Antigens |
| 亚基 | Homodimer. |
| 亚细胞定位 | Lysosome. |
| 相似性 | Belongs to the glycosyl hydrolase 27 family. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | Galactosidase alpha is involved in the hydrolysis of terminal, non reducing alpha D galactose residues in alpha D galactosides, including galactose oligosaccharides, galactomannans and galactohydrolase. Defects in GLA are the cause of Fabry's disease (FD). FD is a rare X-linked sphingolipidosis disease where glycolipid accumulates in many tissues. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. Patients may show ocular deposits, febrile episodes, and burning pain in the extremities. Death results from renal failure, cardiac or cerebral complications of hypertension or other vascular disease. Heterozygous females may exhibit the disorder in an attenuated form, they are more likely to show corneal opacities. |
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GLA Antibody Blocking Peptide(bs-7593P)-500ug
￥880
