alpha-Galactosidase A / GLA抗体

alpha-Galactosidase A / GLA抗体产品信息免疫原 : Recombinant Human alpha-Galactosidase A / GLA protein (Catalog#12078-H08H)

Antibody Type : Mouse Monoclonal Antibody ( Mouse mAb Service Platform )
克隆号 : 2H7G10
抗体宿主 : Mouse IgG2b
缓冲液 : 0.2 μm filtered solution in PBS, 5% trehalose may be added in some batches. Please read the hardcopy of COA or contact our customer service to confirm the formulation.
制备方法 : This antibody was produced from a hybridoma resulting from the fusion of a mouse myeloma with B cells obtained from a mouse immunized with purified, recombinant Human alpha-Galactosidase A / GLA (rh alpha-Galactosidase A / GLA; Catalog#12078-H08H; NP_000940.1; Met 1-Leu 429). The IgG fraction of the cell culture supernatant was purified by Protein A affinity chromatography.
alpha-Galactosidase A / GLA抗体 Background
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.
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