DPAGT1

The enzyme, which is encoded by this gene, is the enzyme involved in the first step of the synthesis of the dolichol phosphate-linked oligosaccharides in the synthesis of the glycoproteins (N-linked glycans) (GlcNAc-1-P transferase, ALG7). Its enzymatic activity is the transfer the N-acetylglucosamine from the sugar nucleotide UDP-GlcNAc (UDP-N-acetylglucosamine), which is a donor substrate, to the dolichol phosphate, as an acceptor substrate. Biosynthesis is performed on the cytoplasmic side of the endoplasmic reticulum membrane. It has become clear that a mutation of this gene results in congenital disorder of glycosylation type Ij.