title: Endocytosis and sorting of glycosphingolipids in sphingolipid storage disease.
abstract: Recent studies on the endocytic itinerary of glycosphingolipids (GSLs) in sphingolipid storage disease (SLSD) fibroblasts have yielded new insights into the mechanisms underlying the endocytosis and intracellular sorting of lipids in normal and disease cells. Here we highlight new data on clathrin-independent endocytosis of GSLs, the involvement of sphingolipid-cholesterol interactions in perturbation of endocytic trafficking, and potential roles for rab proteins in regulation of GSL transport in SLSDs.
authors.affiliation: Dept of Biochemistry and Molecular Biology, Mayo Clinic and Foundation, S.W. Rochester, MN 55905-0001, USA.
authors.forename: David L
authors.initials: DL
authors.lastname: Marks
authors.forename: Richard E
authors.initials: RE
authors.lastname: Pagano
mesh_terms: D000818:Animals; D001692:Biological Transport; D002784:Cholesterol / Q000378:metabolism; D004705:Endocytosis; D006028:Glycosphingolipids / Q000378:metabolism*; D006801:Humans; D008954:Models, Biological; D013106:Sphingolipidoses / Q000378:metabolism* / Q000473:pathology* / Q000628:therapy; D020691:rab GTP-Binding Proteins / Q000378:metabolism
chemical_list: D006028:Glycosphingolipids; D002784:Cholesterol; D020691:rab GTP-Binding Proteins