Ceramide glucosyltransferase


  • Accession: Q16739
  • Swissprot: CEGT_HUMAN
  • Organism: Homo sapiens
  • Gene: UGCG
  • Target class: Enzyme

Drug Relations:

Eliglustat tartrate is a small molecule inhibitor of glucosylceramide synthase that resembles the ceramide substrate for the enzyme. Gaucher disease is caused by a deficiency of the lysosomal enzyme acid beta-glucosidase. Acid beta-glucosidase catalyzes the conversion of the sphingolipid glucocerebroside into glucose and ceramide. The enzymatic deficiency causes an accumulation of glucosylceramide (GL-1) primarily in the lysosomal compartment of macrophages, giving rise to foam cells or "Gaucher cells". Bioactivity details MOA
Miglustat functions as a competitive and reversible inhibitor of the enzyme glucosylceramide synthase, the initial enzyme in a series of reactions which results in the synthesis of most glycosphingolipids. Miglustat helps reduce the rate of glycosphingolipid biosynthesis so that the amount of glycosphingolipid substrate is reduced to a level which allows the residual activity of the deficient glucocerebrosidase enzyme to be more effective (substrate reduction therapy). In vitro and in vivo studies have shown that miglustat can reduce the synthesis of glucosylceramide-based glycosphingolipids. Bioactivity details MOA
edetic acid
A chelating agent that sequesters a variety of polyvalent cations such as CALCIUM. It is used in pharmaceutical manufacturing and as a food additive. Bioactivity details MOA