DPAGT1, NT (GPT, ALG7, DGPT, G1PT, UAGT, UGAT, CDG1J, DPAGT, CDG-Ij, CMSTA2, DPAGT2, UDP-N-acetylglucosamine--dolichyl-Phosphate N-acetylglucosaminephosphotransferase, GlcNAc-1-P Transferase)

The UDP-N-acetylglucosamine-dolichyl-phosphate N-acetyl-glucosaminephosphotransferase (DPAGT1) is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. Mutations in this integral endoplasmic reticulum (ER) membrane protein enzyme belongs to the glycosyltransferase family 4 results in the congenital disorder of glycosylation type Ij with symptoms such as severe hypotonia, medically intractable seizures, mental retardation, microcephaly, and exotropia. Recent experiments have shown that DPAGT1 is a target of the Wnt/beta-catenin signaling...