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重组人alpha-Galactosidase A/GLAProtein(C-6His)

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产品名称: 重组人alpha-Galactosidase A/GLAProtein(C-6His)
产品型号: PN100053-50ug
产品展商: PERFEMIKER
产品价格: 2800.00 元
会员价格: 0.00 元
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简单介绍

重组人alpha-Galactosidase A/GLAProtein(C-6His) 货号:PN100053 规格:50ug; 标签:C-6His; 来源:Human; 表达宿主:Human cells


重组人alpha-Galactosidase A/GLAProtein(C-6His)  的详细介绍

名称:重组人alpha-Galactosidase A/GLAProtein(C-6His);



产品说明(Description)
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. 
Accession #: P06280
Known as: Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA

制剂(Formulation)
Supplied as a 0.2 μm filtered solution of 20mM TrisHCl, 150mM NaCl, pH 8.0.

质量控制(Quality Control) 
Purity: Greater than 95% as determined by reducing SDS-PAGE.    
Endotoxin:    Less than 0.1 ng/ug (1 IEU/ug) as determined by LAL test.

复溶(Reconstitution)

保存(Storage)
Store at < -20°C, stable for 6 months after receipt.Please minimize freeze-thaw cycles.   

背景(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.

电泳(SDS-PAGE)

FOR RESEARCH USE ONLY