Reactome: A Curated Pathway Database
All 7 results
Pathways (4) Reactions (1) Proteins (1) Others (1)
Protein: UniProt:P04062 GBA (Homo sapiens)
Last changed: 2014-11-26 10:20:21

Pathway: Metabolism (Homo sapiens)
Metabolic processes in human cells generate energy through the oxidation of molecules consumed in the diet and mediate the synthesis of diverse essential molecules not taken in the diet as well as the inactivation and elimination of toxic ones generated endogenously or present in the extracellular environment. The processes of energy metabolism can be classified into two groups according to whether the
Last changed: 2014-11-21 19:49:01

Pathway: Metabolism of lipids and lipoproteins (Homo sapiens)
Lipids are hydrophobic but otherwise chemically diverse molecules that play a wide variety of roles in human biology. They include ketone bodies, fatty acids, triacylglycerols, phospholipids and sphingolipids, eicosanoids, cholesterol, bile salts, steroid hormones, and fat-soluble vitamins. They function as a major source of energy (fatty acids, triacylglycerols, and ketone bodies), are major constitue
Last changed: 2014-11-21 19:49:01

Pathway: Sphingolipid metabolism (Homo sapiens)
Sphingolipids are derivatives of long chain sphingoid bases such as sphingosine (trans-1,3-dihydroxy 2-amino-4-octadecene), an 18-carbon unsaturated amino alcohol which is the most abundant sphingoid base in mammals. Amide linkage of a fatty acid to sphingosine yields ceramides. Esterification of phosphocholine to ceramides yields sphingomyelin, and ceramide glycosylation yields glycosylceramides. Intr
Last changed: 2014-11-21 19:49:01

Pathway: Glycosphingolipid metabolism (Homo sapiens)
The steps involved in the synthesis of glycosphingolipids (sphingolipids with one or more sugars attached) are annotated here (Gault et al. 2010)
Last changed: 2014-11-21 19:49:01

Reaction: Glucosylceramidase cleaves the glucosidic bond of glucocerebroside to form ceramide (Homo sapiens)
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 glycolip
Last changed: 2014-11-21 14:40:22

Complex: GBA:SAPC [lysosomal membrane] (Homo sapiens)
Last changed: 2011-10-31 14:18:02