Recombinant Human alpha-Galactosidase A/GLA,BR

产品属性 Product Attributes

基本信息

别名： Alpha-Galactosidase A;Alpha-D-Galactosidase A;Alpha-D-Galactoside Galactohydrolase;Melibiase;Agalsidase;GLA
规格: 10ug ; 50ug ; 500ug ; 1mg
外观(性状)： Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.
纯度: ≥95% as determined by reducing SDS-PAGE.
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.

产品信息

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.
来源： Human Cells

其它标识

内毒素: Less than 0.1 ng/ug(1 EU/ug)as determined by LAL test.

产品介绍 Product Introduction

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注意事项 Considerations

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