Gene:
asparagine-linked glycosylation 6 homolog (yeast, alpha-1,3-glucosyltransferase)
Description:
This gene encodes the enzyme Man(9)GlcNAc(2)-PP-Dol alpha-1,3-glucosyltransferase. This enzyme participates in the glucosylation of the oligomannose core. The addition of glucose residues to the oligomannose core is necessary to ensure substrate recognition, and therefore, effectual transfer of the oligomannose core to the nascent glycoproteins. Mutations in this gene result in an accumulation of glucosylated lipid-linked oligosaccharides which leads to carbohydrate-deficient glycoprotein syndrome.