Mannose metabolism and carbohydrate deficient glycoprotein syndrome type I

Monosaccharide mannose is a constituent of glycoproteins bearing N-linked oligosaccharide chains and the glycolipid anchors that enable proteins without membrane spanning domains to associate with cellular membranes. With, on the one hand, the description of Carbohydrate-Deficient Glycoprotein Syndrome (CDGS), a group of metabolic diseases in which glycoprotein biosynthesis is severely impaired, and, on the other, the recent identification of specific mannose transporters, our view of the metabolism of mannose, and its relationship with glucose metabolism is changing. These new insights into the metabolism or mannose has led to the proposal of therapies for cer tain types cif CDGS.