Recombinant Human GLA (C-6His)
- 货号：EPT291
- 价格：￥26,400.00 元
- 点击：942
产品介绍
基本信息
| 规格 | 1mg |
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| 表达宿主 | Human Cells |
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| 标签 | C-6His |
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物理性质
| 分子量 | 46.39 KDa |
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| 表观分子量 | 50-60 KDa, reducing conditions |
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| 形态 | Supplied as a 0.2 μm filtered solution of 20mM Tris-HCl, 150mM NaCl, pH 8.0. |
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| 纯度 | Greater than 95% as determined by reducing SDS-PAGE. |
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活性与内毒素
| 内毒素水平 | Less than 0.1 ng/µg (1 EU/µg) as determined by LAL test. |
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| 活性 | Measured by its ability to hydrolyze 4-Nitrophenyl -alpha -D-galactopyranoside. The specific activity is 2835 pmol/min/µg. |
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储存与运输
| 储存方法 | 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. |
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| 运输条件 | The product is shipped on dry ice/polar packs.
Upon receipt, store it immediately at the temperature listed below. |
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详细说明
| 背景说明 | 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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