Glucosylceramide synthase and glycosphingolipid synthesis

Abstract

In mammalian cells, there are two major classes of sphingolipids---sphingomyelin and glycosphingolipids (GSLs)--both of which are synthesized from the hydrophobic molecule ceramide. The synthesis of most GSLs begins with glucosylation of ceramide to form glucosylceramide (GlcCer), which, in turn, serves as the source of 300-400 GSLs. Although most of these GSLs have been characterized chemically, the biological functions of ceramide glycosylation and GSLs still remain enigmatic. The recent description of a GSL-deficient cell line and isolation of cDNA for GlcCer synthase provide new insights into GSL functions.