Mouse GLA / Alpha-galactosidase A Protein|產品詳情|價格|參數

GLA / Alpha-galactosidase A蛋白產品信息

別名: Gla
表達方式: A DNA sequence encoding the mouse Gla (Q8BGZ6) (Met1- Arg421) was expressed with a C-terminal polyhistidine tag.
種屬: Mouse
表達宿主: Human Cells

GLA / Alpha-galactosidase A Protein QC Testing

純度: >95% as determined by SDS-PAGE SDS-PAGE
內**: < 1.0 EU per μg of the protein as determined by the LAL method
穩定性: Samples are stable for up to twelve months from date of receipt at -70℃
預測N端: Leu 34
分子量: The recombinant mouse Gla comprises 399 amino acids and has a predicted molecular mass of 45.6 kDa. The apparent molecular mass of the protein is approximately 46-52 kDa in SDS-PAGE under reducing conditions due to glycosylation.
緩沖液: Lyophilized from sterile PBS, pH7.4.
Normally 5 % - 8 % trehalose and mannitol are added as protectants before lyophilization. Specific concentrations are included in the hardcopy of COA.
Please contact us for any concerns or special requirements.

GLA / Alpha-galactosidase A Protein Usage Guide

儲存方法: Store it under sterile conditions at -70℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
Reconstitution: A hardcopy of COA with reconstitution instruction is sent along with the products. Please refer to it for detailed information.

GLA / Alpha-galactosidase A蛋白背景綜述

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.

參考文獻

Koide T. et al., 1990, FEBS Lett. 259: 353-6.
Yang C-C. et al., 2003, Clin. Genet. 63: 205-9.
Verovnik F. et al., 2004, Eur J Hum Genet. 12: 678-81.
Nance CS. et al., 2006, Arch Neurol. 63: 453-7.

規格：20ug

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