title: Glucosylceramide synthase: assay and properties.
abstract: Glucosylceramide synthesis is a key step in the formation of most mammalian glycosphingolipids. The expanding number of cellular functions that may be glycosphinolipid dependent and the identification of this glucosylceramide synthase as a potential therapeutic target for several sphingolipid storage disorders necessitate the availability of a reliable assay for glucosylceramide synthase. Coupled with the recent sequencing of this enzyme, the liposome-based assay utilizing a single extraction step should aid in the understanding of this critical early pathway in glycosphingolipid formation.
authors.affiliation: Department of Internal Medicine, University of Michigan Medical School, Ann Arbor 48109-0676, USA.
authors.forename: J A
authors.initials: JA
authors.lastname: Shayman
authors.forename: A
authors.initials: A
authors.lastname: Abe
mesh_terms: D002413:Cations, Divalent; D002518:Ceramides / Q000378:metabolism; D004790:Enzyme Induction; D005964:Glucosyltransferases / Q000032:analysis* / Q000037:antagonists & inhibitors / Q000378:metabolism*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008081:Liposomes / Q000378:metabolism; D013106:Sphingolipidoses / Q000628:therapy; D013107:Sphingolipids / Q000138:chemical synthesis; D013110:Sphingosine / Q000031:analogs & derivatives / Q000138:chemical synthesis; D013379:Substrate Specificity; D014532:Uridine Diphosphate Glucose / Q000378:metabolism
chemical_list: C066099:2,3-N-octanoylsphingosine; D002413:Cations, Divalent; D002518:Ceramides; D008081:Liposomes; D013107:Sphingolipids; C401331:glucosyloctanoylsphingosine; D005964:Glucosyltransferases; C023246:ceramide glucosyltransferase; D013110:Sphingosine; D014532:Uridine Diphosphate Glucose