With the molecular identification of genes encoding many additional enzymes of sphingolipid metabolism, it is now appreciated that several inherited, non-lysosomal disorders of sphingolipid metabolism exist and involve alteration of SPT, individual CerSs, fatty acid alpha hydroxylase, alkaline CDases, S1P lyase, CERT1, Des1/DEGS1, and others (159), including congenital disorders of glycosylation (160). Here, DEGS1 is linked to lysosomal storage disease.