title: [Glycolipids of the cell surface--biochemistry of their decomposition].
abstract: Glycosphingolipids (GSL) form cell-type-specific patterns on the surface of eukaryotic cells. For the maintenance of these patterns, biosynthesis, intracellular transport, and degradation of GSL are thought to proceed in a coordinated fashion. After transport from the plasma membrane to the lysosomes the degradation of GSL requires protein cofactors to solubilize the membrane-bound substrates of the degradative enzymes. Inborn errors of metabolism leading to defective hydrolases or activator proteins give rise to sphingolipid storage diseases; in some cases the residual enzyme activity can be correlated with the clinical manifestations.
mesh_terms: D000818:Animals; D002236:Carbohydrate Conformation; D002240:Carbohydrate Sequence; D002462:Cell Membrane / Q000378:metabolism* / Q000648:ultrastructure; D004705:Endocytosis; D005057:Eukaryotic Cells; D006017:Glycolipids / Q000096:biosynthesis / Q000737:chemistry / Q000378:metabolism*; D006028:Glycosphingolipids / Q000096:biosynthesis / Q000737:chemistry / Q000378:metabolism*; D006801:Humans; D016464:Lysosomal Storage Diseases / Q000378:metabolism; D008247:Lysosomes / Q000378:metabolism; D008563:Membrane Lipids / Q000096:biosynthesis / Q000737:chemistry / Q000378:metabolism*; D008961:Models, Structural; D008969:Molecular Sequence Data
chemical_list: D006017:Glycolipids; D006028:Glycosphingolipids; D008563:Membrane Lipids
vernacular_title: Glykolipide der Zelloberfläche--Biochemie ihres Abbaus.