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Gene Review

DPAGT1  -  dolichyl-phosphate (UDP-N...

Homo sapiens

Synonyms: ALG7, CDG-Ij, CDG1J, CMSTA2, D11S366, ...


This gene is homolog to the yeast gene ALG7, and it is the initial enzyme in the synthesis of the N-glycan precursor.


Functions (from Uniprot)

Catalyzes the initial step in the synthesis of dolichol-P-P-oligosaccharides.


Disease relevance of DPAGT1

  • In the present study we describe the effects of all-trans-retinoic acid (RA) on the levels of GPT protein and enzymic activity, and on the transcription rate of the GPT gene, in mouse P19 teratocarcinoma cells [1].
  • Serum markers of liver function [GOT, GPT, gamma-GTP, total bile acids (TBA)], and serum markers of liver fibrosis [hyaluronic acid (HA), type IV collagen (C-IV)], were measured in both groups at the beginning of the study, and at 1, and 3 years after the beginning of the study and the results compared statistically [2].
  • Postoperative liver functions showed no marked derangement; the mean peak GPT was 221 U and the mean peak total bilirubin 2.3 mg d/l. Local cooling minimizes the risk of ischemia/reperfusion injury in this very vulnerable population, yet gives the surgeon adequate time to perform a challenging resection in a bloodless field [3].
  • There was no statistical difference in the toxicity grading of hemoglobin, glutamic oxaloacetic transaminase/glutamic pyruvic transaminase (GOT/GPT) and stomatitis/ gastritis between the three groups of patients [4].

High impact information on DPAGT1


Biological context of DPAGT1


Anatomical context of DPAGT1

  • In addition, GPT activity in membranes from RA-treated P19 cells exhibited approx. 4-fold increased resistance to tunicamycin compared with activity in membranes from untreated control cells, demonstrating that resistance to tunicamycin is correlated with induced GPT activity [1].
  • This difference suggests that dolichyl phosphate may be limiting for GlcNAc-1-P transferase activity in endoplasmic reticulum of the older animals [12].
  • In vitro enzymatic analysis of microsomal fractions from the cultured cells indicated that oligosaccharyltransferase activity is normal, but the GPT activity is reduced to approximately 10% of normal levels while parents have heterozygous levels [10].
  • The amount of dolichyl phosphate and GlcNAc-1-P transferase activity in CDG syndrome fibroblasts were similar to those in normal fibroblasts, suggesting that CDG syndrome may not be due to a deficiency of a biosynthetic enzyme for dolichol-oligosaccharide intermediates, but to a metabolic error in assembly of asparagine-linked oligosaccharide [13].
  • To initiate the molecular characterization of ALG7 involvement in mammalian growth and differentiation, we have used the postnatally developing hamster submandibular gland (SMG) as an experimental paradigm [14].

Associations of DPAGT1 with chemical compounds


Other interactions of DPAGT1


Analytical, diagnostic and therapeutic context of DPAGT1


  1. Regulation of UDP-N-acetylglucosamine:dolichyl-phosphate N-acetylglucosamine-1-phosphate transferase by retinoic acid in P19 cells. Meissner, J.D., Naumann, A., Mueller, W.H., Scheibe, R.J. Biochem. J. (1999) [Pubmed]
  2. Inchin-ko-to prevents medium-term liver fibrosis in postoperative biliary atresia patients. Tamura, T., Kobayashi, H., Yamataka, A., Lane, G.J., Koga, H., Miyano, T. Pediatr. Surg. Int. (2007) [Pubmed]
  3. Hepatic resection under in situ hemihepatic hypothermic perfusion with hepatoprotective agents. Yamanaka, N., Okamoto, E., Furukawa, K., Oriyama, T., Fujimoto, J., Kanno, H., Kawamura, E., Tanaka, T., Tomoda, H., Ichikawa, N. Hepatogastroenterology (1995) [Pubmed]
  4. Feasibility of adjuvant chemotherapy for breast cancer patients. Imoto, S. Jpn. J. Clin. Oncol. (1997) [Pubmed]
  5. The 63-kilobase circular amplicon of tunicamycin-resistant Leishmania amazonensis contains a functional N-acetylglucosamine-1-phosphate transferase gene that can be used as a dominant selectable marker in transfection. Liu, X., Chang, K.P. Mol. Cell. Biol. (1992) [Pubmed]
  6. Coupling of the dolichol-P-P-oligosaccharide pathway to translation by perturbation-sensitive regulation of the initiating enzyme, GlcNAc-1-P transferase. Gao, N., Lehrman, M.A. J. Biol. Chem. (2002) [Pubmed]
  7. Role of the carboxyl terminus in stable expression of hamster UDP-GlcNAc:dolichol-P GlcNAc-1-P transferase. Zara, J., Lehrman, M.A. J. Biol. Chem. (1994) [Pubmed]
  8. Both potential dolichol recognition sequences of hamster GlcNAc-1-phosphate transferase are necessary for normal enzyme function. Datta, A.K., Lehrman, M.A. J. Biol. Chem. (1993) [Pubmed]
  9. Evidence that the hamster tunicamycin resistance gene encodes UDP-GlcNAc:dolichol phosphate N-acetylglucosamine-1-phosphate transferase. Zhu, X., Zeng, Y., Lehrman, M.A. J. Biol. Chem. (1992) [Pubmed]
  10. Deficiency of UDP-GlcNAc:Dolichol Phosphate N-Acetylglucosamine-1 Phosphate Transferase (DPAGT1) causes a novel congenital disorder of Glycosylation Type Ij. Wu, X., Rush, J.S., Karaoglu, D., Krasnewich, D., Lubinsky, M.S., Waechter, C.J., Gilmore, R., Freeze, H.H. Hum. Mutat. (2003) [Pubmed]
  11. Genomic structure of the human UDP-GlcNAc:dolichol-P GlcNAc-1-P transferase gene. Regis, S., Dagnino, F., Caroli, F., Filocamo, M. DNA Seq. (2002) [Pubmed]
  12. Dolichol-linked glycoprotein synthesis in developing mammalian brain: maturational changes of the N-acetylglucosaminylphosphotransferase. Volpe, J.J., Sakakihara, Y., Ishii, S. Brain Res. (1987) [Pubmed]
  13. Major defect of carbohydrate-deficient-glycoprotein syndrome is not found in the synthesis of dolichyl phosphate or N-acetylglucosaminyl-pyrophosphoryl-dolichol. Yasugi, E., Nakasuji, M., Dohi, T., Oshima, M. Biochem. Biophys. Res. Commun. (1994) [Pubmed]
  14. Developmental regulation and tissue-specific expression of hamster dolichol-P-dependent N-acetylglucosamine-1-P transferase (GPT). Mota, O.M., Huang, G.T., Kukuruzinska, M.A. Biochem. Biophys. Res. Commun. (1994) [Pubmed]
  15. Properties of a soluble polyprenyl phosphate. UDP-D-N-acetylglucosamine N-acetylglucosamine-1-phosphate transferase. Villemez, C.L., Carlo, P.L. J. Biol. Chem. (1980) [Pubmed]
  16. A large deletion on chromosome 11 in acute intermittent porphyria. Di Pierro, E., Besana, V., Moriondo, V., Brancaleoni, V., Tavazzi, D., Casalgrandi, G., Ventura, P., Rocchi, E., Cappellini, M.D. Blood Cells Mol. Dis. (2006) [Pubmed]
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