| 产品名称: |
Recombinant Human GLA (C-6His) |
| 产品描述: |
Recombinant Human Alpha-Galactosidase is produced by our Mammalian expression system and the target gene encoding Leu32-Leu429 is expressed with a 6His tag at the C-terminus. |
| 蛋白编号: |
P06280 |
| 分子量: |
46.39 KDa |
| 表观分子量: |
50-60 KDa, reducing conditions |
| 产品纯度: |
Greater than 95% as determined by reducing SDS-PAGE. |
| 内毒素: |
Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
| 生物活性: |
Measured by its ability to hydrolyze 4-Nitrophenyl -alpha -D-galactopyranoside. The specific activity is 2835 pmol/min/µg. |
| 储存条件: |
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. |
| 运输条件: |
The product is shipped on dry ice/polar packs.
Upon receipt, store it immediately at the temperature listed below. |
| 研究背景: |
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. |
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