Reactome: A Curated Pathway Database

Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (R-HSA-1605591)

Species Homo sapiens

Summation

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).

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Additional Information
Compartment lysosomal membrane , lysosomal lumen
Components of this entry
Input entries
Output entries
Catalyst Activity
PhysicalEntity Activity Active Units
GBA:SAPC glucosylceramidase activity (0004348)