Article
Catalytic mechanism of human alpha-galactosidase.
The Journal of biological chemistry - 5 Feb 2010
Guce Abigail I, Clark Nathaniel E, Salgado Eric N, Ivanen Dina R, Kulminskaya Anna A, Brumer Harry, Garman Scott C
Abstract excerpt
The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL A; E.C. 3.2.1.22) is responsible for the breakdown of alpha-galactosides in the lysosome. Defects in human alpha-GAL lead to the development of Fabry disease, a lysosomal storage disorder characterized by the buildup of alpha-galactosylated substrates in the tissues. alpha-GAL is an active target of clinical research: there are currently two...
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