GLA/alpha-Galactosidase A Protein, Human, Recombinant (His)

GLA/alpha-Galactosidase A Protein, Human, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 46.8 kDa and the accession number is P06280.

规格	价格/CNY		数量
50 μg	￥ 3,170	现货
100 μg	￥ 5,610	5日内发货
200 μg	￥ 9,920	5日内发货
500 μg	￥ 20,980	5日内发货

规格

价格/CNY

货期

数量

50 μg

￥ 3,170

现货

100 μg

￥ 5,610

5日内发货

200 μg

￥ 9,920

5日内发货

500 μg

￥ 20,980

5日内发货

生物活性	Testing in progress
产品描述	GLA/alpha-Galactosidase A Protein, Human, Recombinant (His) is expressed in HEK293 mammalian cells with His tag. The predicted molecular weight is 46.8 kDa and the accession number is P06280.

种属	Human
表达系统	HEK293 Cells
标签	C-His
蛋白编号	P06280
别名	GLA/α-Galactosidase A Protein, galactosidase, α, galactosidase, alpha, GALA
蛋白构建	The Human GLA (NP_000160.1) (Met 1-Leu 429) was fused with a polyhistidine tag at the C-terminus.
蛋白纯度	> 97 % as determined by SDS-PAGE
分子量	46.8 kDa (predicted)

内毒素	< 1.0 EU/μg of the protein as determined by the LAL method.
缓冲液	Lyophilized from a solution filtered through a 0.22 μm filter, containing 50 mM Tris, 150 mM NaCl, pH 7.5. Typically, a mixture containing 5% to 8% trehalose, mannitol, and 0.01% Tween 80 is incorporated as a protective agent before lyophilization.
复溶方法	A Certificate of Analysis (CoA) containing reconstitution instructions is included with the products. Please refer to the CoA for detailed information.
存储	It is recommended to store recombinant proteins at -20°C to -80°C for future use. Lyophilized powders can be stably stored for over 12 months, while liquid products can be stored for 6-12 months at-80℃. For reconstituted protein solutions, the solution can be stored at -20°c to -80'c for at least 3 months. Please avoid multiple freeze-thaw cycles and store products in aliquots.
运输方式	In general, Lyophilized powders are shipping with blue ice.
研究背景	Alpha-galactosidase A, also known as Alpha-D-galactoside galactohydrolase, Alpha-D-galactosidase A, Melibiase and GLA, is a member of the glycosyl hydrolase 27 family. GLA is used as a long-term enzyme replacement therapy in patients with a confirmed diagnosis of Fabry disease. Defects in GLA are the cause of Fabry disease (FD) which is a rare X-linked sphingolipidosis disease where 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. Clinical recognition in males results from characteristic skin lesions (angiokeratomas) over the lower trunk. 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. Deficiency of GLA leads to the accumulation of glycosphingolipids in the vasculature leading to multiorgan pathology. In addition to well-described microvascular disease, deficiency of GLA is also characterized by premature macrovascular events such as stroke and possibly myocardial infarction.