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產(chǎn)品名稱

Galactosidase, alpha (GLA) (AA 32-429) protein (His tag) from ANTIBODIES-ONLINE GmbH

產(chǎn)品貨號(hào)

ABIN1095992

產(chǎn)品價(jià)格

現(xiàn)貨詢價(jià)，電話：010-67529703

產(chǎn)品規(guī)格

10 μg

產(chǎn)品品牌

ANTIBODIES-ONLINE GmbH

產(chǎn)品概述

產(chǎn)品詳情

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Product Name	Galactosidase, alpha (GLA) (AA 32-429) protein (His tag)
Description	alpha-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. alpha-Galactosidase A can hydrolyze terminal alpha-galactosyl moieties from glycolipids and glycoproteins and catalyze the hydrolysis of melibiose into galactose and glucose. Defects alpha-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.Alternative Names: Alpha-Galactosidase A, Alpha-D-Galactosidase A, Alpha-D-Galactoside Galactohydrolase, Melibiase, Agalsidase, GLA
Size	10 μg
Concentration	n/a
Applications	n/a
Other Names	MGC130872\|SMU.877\|SCF11.21\|AO090005000217\|GALA\|zgc:101584\|Ags\|alpha-GAL\|AW555139\|GM130\|mKIAA4150
Gene, Accession, CAS #	Gene ID: 2717
Catalog #	ABIN1095992
Price
Order / More Info	Galactosidase, alpha (GLA) (AA 32-429) protein (His tag) from ANTIBODIES-ONLINE GmbH
Product Specific References	n/a
產(chǎn)品資料

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