Source:http://www.reactome.org/biopax/48887BiochemicalReaction4631
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Authored: Jassal, B, 2011-09-21,
Edited: Jassal, B, 2011-09-21,
Human glucosylceramidase (GBA) hydrolyses the glucosidic bond of glucocerebrosides to form ceramide (Dinur et al. 1986). GBA requires a low weight, non-enzymatic protein (one of the sphingolipids activator proteins) called Saposin-C (SAP-C) which acts with GBA to form an activated complex (Salvioli et al. 2000). Defects in GBA are the cause of Gaucher disease (GD) (MIM:230800), the most common glycolipid storage disorder, characterized by storage of glucocerebroside in the liver, spleen, and marrow (Beutler & Gelbart 1996).,
Reviewed: Stephan, R, 2011-10-31
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Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide
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3.2.1.45
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