Activator proteins and topology of lysosomal sphingolipid catabolism

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.