EC Number   |
General Information |
Reference |
|---|
    2.7.8.15 | malfunction |
N-glycan biosynthesis can be inhibited by disruption of the first committed enzyme, DPAGT1 |
759290 |
| 2.7.8.15 | malfunction |
pathogenic mutations in DPAGT1 are manifested as two possible phenotypes: congenital disorder of glycosylation DPAGT1-CDG (also known as CDG-Ij), and limb-girdle congenital myasthenic syndrome (CMS) with tubular aggregates, analysis of DPAGT1 transcriptional profiles and GTP levels in patient-derived fibroblast. enzyme mutations affect the splicing process, the stability of GTP, or the ability of this protein to correctly localise in the endoplasmic reticulum membrane |
760027 |
| 2.7.8.15 | metabolism |
UDP-N-acetylglucosamine-dolichylphosphate N-acetylglucosamine phosphotransferase (GPT) is involved in an initial step in the N-glycosylation pathway |
760027 |
| 2.7.8.15 | more |
catalytic mechanism of DPAGT1, overview |
759290 |
| 2.7.8.15 | physiological function |
AglH is essential for viability in Sulfolobus acidocaldarius. AglH is able to replace the endogenous GlcNAc-1-phosphotransferase activity of Alg7 in a conditional lethal Saccharomyces cerevisiae strain, in which the first step of the eukaryal protein N-glycosylation process is repressed |
-, 761051 |
| 2.7.8.15 | physiological function |
DPAGT1 is an integral membrane protein localized in the endoplasmic reticulum (ER) that catalyzes the transformation from UDP-GlcNAc to N-acetyl-D-glucosaminyl-diphosphodolichol. Anchored N-acetyl-D-glucosaminyldiphosphodolichol in the ER membrane is modified by sequential glycosyltransferases to form dolichol-linked oligosaccharide precursors that are transferred to selected asparagine residues of polypeptide chains by oligosaccharyltransferase. DPAGT1 catalyzes N-glycosylation of beta-catenin and E-cadherin |
759290 |
| 2.7.8.15 | physiological function |
gene disruption or deletion result in larval lethality, defects in oogenesis and oocyte-to-embryo transition. Endomitotic oocytes, abnormal fusion of pronuclei, abnormal AB cell rotation, disruption of permeation barriers of eggs, and abnormal expression of chitin and chitin synthase in oocytes and eggs are the typical phenotypes observed |
761219 |
| 2.7.8.15 | physiological function |
GPT is involved in glycosylation of proteins on asparagine amino acids |
703322 |
| 2.7.8.15 | physiological function |
the endoplasmic reticulum (ER)-resident transmembrane protein catalyses the transfer of N-acetylglucosamine from cytosolic UDP-N-acetylglucosamine to dolichol-phosphate, which is also located in the ER membrane. The result is the formation of dolichol-diphosphate-N-acetylglucosamine, the carrier of the sugars that are finally attached to proteins in glycosylation |
760027 |
