DPAGT1 encoded by this gene is an enzyme that catalyzes the first step in the dolichol-linked oligosaccharide pathway for glycoprotein biosynthesis. This enzyme belongs to the glycosyltransferase family 4. This protein is an integral membrane protein of the endoplasmic reticulum. The congenital disorder of glycosylation type Ij is caused by mutation in the gene encoding this enzyme.
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Protein Aliases: dolichyl-phosphate (UDP-N-acetylglucosamine) acetylglucosaminephosphotransferase 1 (GlcNAc-1-P transferase); dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P tra; dolichyl-phosphate (UDP-N-acetylglucosamine) N-acetylglucosaminephosphotransferase 1 (GlcNAc-1-P transferase); dolichyl-phosphate alpha-N-acetylglucosaminephosphotransferase 2; dolichyl-phosphate alpha-N-acetylglucosaminyltransferase; G1PT; GlcNAc-1-P transferase; GlcNAc-1-P transferase 1; N-acetylglucosamine-1-phosphate transferase; UDP-GlcNAc:dolichyl-phosphate N-acetylglucosaminephosphotransferase; UDP-N-acetylglucosamine lysosomal enzyme N-acetylglucosamine phosphotransferase; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase; UDP-N-acetylglucosamine--dolichyl-phosphate N-acetylglucosaminephosphotransferase 1
Gene Aliases: ALG7; AU021132; CDG-Ij; CDG1J; CMS13; CMSTA2; D11S366; DGPT; DPAGT; DPAGT1; DPAGT2; G1PT; Gnpta; GPT; H2afx; UAGT; UGAT