UGCG and lysosomal storage disease: Inhibitors of uridine diphosphate (UDP)-glucosylceramide synthase (UDP-glucose: ceramide glucosyltransferase) that catalyses the first step in glycosphingolipid biosynthesis (synthesis of glucosylceramide) are being administered to patients with several sphingolipid lysosomal storage disorders as part of substrate reduction therapy (22,23).