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产品名称
起订量
单价(含增值税)
库存(PCS)
起订数量
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Recombinant Human alpha-Galactosidase A/GLA48T/96T￥ 22001
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Recombinant Human alpha-Galactosidase A/GLAGV-P01642-10ug￥ 1310.001
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Recombinant Human alpha-Galactosidase A/GLAGV-P01642-50ug￥ 4000.001
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Recombinant Human alpha-Galactosidase A/GLAGV-P01642-500ug￥ 13520.001
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Recombinant Human alpha-Galactosidase A/GLAGV-P01642-1mg￥ 18800.001
产品简介
| Known as | Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA |
| Derived from | Human Cells |
| Purity | Greater than 95% as determined by reducing SDS-PAGE. |
| Formulation | Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0. |
| Storage | Store at ≤-70°C, stable for 6 months after receipt.Store at ≤-70°C, stable for 3 months under sterile conditions after opening.Please minimize freeze-thaw cycles. |
| Endotoxin | Less than 0.1 ng/ug (1 EU/ug) as determined by LAL test. |
| Background | 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. |
| 规格 | 10ug50ug500ug1mg |
注意：
1.本产品仅供科研使用。请勿用于医药、临床诊断或治疗，食品及化妆品等用途。请勿存放于普通住宅区。
2.为了您的安全和健康，请穿好实验服并佩戴一次性手套和口罩操作。
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