重组人α-半乳糖苷酶

特别提示：包括重组人α-半乳糖苷酶在内，本公司的所有产品仅可用于科研实验，严禁用于临床医疗及其他非科研用途！

产品名称：重组人α-半乳糖苷酶
英文名称：Recombinant Human alpha-Galactosidase
产品规格：10μg|50μg|500μg|1mg

本品由我们的哺乳动物细胞表达系统制备而成，目的基因编码的Leu32-Leu429在C端含有His标签。

α-Galactosidase质量控制：＞95%(还原性SDS-PAGE)

α-Galactosidase制剂：液体

α-Galactosidase保存：收到货后请置于-20℃，可保存6个月，避免反复冻融。

关于α-Galactosidase：
α-Galactosidase A is a homodimeric glycoprotein that belongs to the glycosyl hydrolase 27 family. It is a lysosomal enzyme and used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. α-Galactosidase A can hydrolyze terminal α-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects α-Galactosidase A are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease with glycolipid accumulates in many tissues. The disease consists of an inborn error of glycosphingolipid catabolism. FD patients show systemic accumulation of globotriaoslyceramide (Gb3) and related glycosphingolipids in the plasma and cellular lysosomes throughout the body. 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.