[Lipid-protein interactions: mechanisms of enzymatic glycolipid catabolism and their genetic restrictive escapes].

Investigations of the genetic basis of ganglioside catabolism have led to the characterisation of two types of lipid-enzyme interaction: a) Breakdown of membrane-bound glycolipids as far as catalysed by membrane-bound enzymes is regulated by the membrane itself. b) The degradation of micelle-forming glycolipids by water-soluble lysosomal enzymes is facilitated by cofactors known as activator proteins. A genetic defect in an activator protein can be just as fatal as the lack of the enzyme itself.