New Approaches in the Study of Ganglioside Metabolism

Abstract
It is generally accepted that gangliosides are biosynthesized in the Golgi apparatus and then move to the plasma membrane which is the main site of their cellular location. 1–3 The process of ganglioside biosynthesis consists of sequential additions of monosaccharide units to a starting precursor (ceramide or psychosine) catalyzed by specific glycosyltransferases.1, 2, 4, 5 Gangliosides are degraded in the lysosomes, where several glycohydrolases remove sequentially the individual saccharide units and the formed ceramide is split by ceramidase into sphingosine and fatty acid.6–8 In the case of nerve cells the process of both biosynthesis and degradation of gangliosides is assumed to occur primarily in the perikaryon.7–9 Transport of gangliosides to and from the plasma membranes is mediated by fast axonal flow.9, 10