title: Activator proteins and topology of lysosomal sphingolipid catabolism.
abstract: The lysosomal degradation of several sphingolipids by acid hydrolases is dependent on small non-enzymic cofactors, called sphingolipid activator proteins some of which have been identified as sphingolipid binding proteins. This review summarizes the information available on the structure, function, biosynthesis, gene organization and pathobiochemistry of the known sphingolipid activator proteins. It also offers models for their mode of action and for the topology of lysosomal digestion of glycolipids.
authors.affiliation: Institute for Organic Chemistry and Biochemistry, University of Bonn, Germany.
authors.forename: W
authors.initials: W
authors.lastname: Fürst
authors.forename: K
authors.initials: K
authors.lastname: Sandhoff
mesh_terms: D000818:Animals; D002240:Carbohydrate Sequence; D049289:G(M2) Activator Protein; D005786:Gene Expression Regulation; D006023:Glycoproteins / Q000737:chemistry / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D008247:Lysosomes / Q000378:metabolism*; D008954:Models, Biological; D008969:Molecular Sequence Data; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism*; D011506:Proteins / Q000378:metabolism*; D049231:Saposins; D049128:Sphingolipid Activator Proteins; D013107:Sphingolipids / Q000737:chemistry / Q000378:metabolism*
chemical_list: D049289:G(M2) Activator Protein; D006023:Glycoproteins; C483345:PSAP protein, human; D011498:Protein Precursors; D011506:Proteins; D049231:Saposins; D049128:Sphingolipid Activator Proteins; D013107:Sphingolipids