Recombinant Human GLA (C-6His) [EPT291] | ナミキ商事株式会社

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サプライヤー: ELK Biotechnology
カタログNo.: EPT291
製品名称: Recombinant Human GLA (C-6His)
タンパク質名: 46.39 KDa
Species: -
HOST: Alpha-Galactosidase A; Alpha-D-Galactosidase A; Alpha-D-Galactoside Galactohydrolase; Melibiase; Agalsidase; GLA

Synonyms	-
Characteristics	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.
Endotoxin Level	Less than 0.1 ng/?g (1 EU/?g) as determined by LAL test.
Purity	Greater than 95% as determined by reducing SDS-PAGE.
Description	-
Reference	-
URL	-

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