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GNPAT  -  glyceronephosphate O-acyltransferase

Homo sapiens

Synonyms: Acyl-CoA:dihydroxyacetonephosphateacyltransferase, DAP-AT, DAPAT, DHAP-AT, DHAPAT, ...
 
 
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Disease relevance of GNPAT

 

High impact information on GNPAT

 

Chemical compound and disease context of GNPAT

 

Biological context of GNPAT

 

Anatomical context of GNPAT

  • These data demonstrate that a defect in the gene that codes for peroxisomal DHAPAT is the primary lesion in the NRel-4 cell line and that the peroxisomal DHAPAT is essential for the biosynthesis of plasmalogens in animal cells [11].
  • Alkyl-dihydroxyacetone phosphate synthase and dihydroxyacetone phosphate acyltransferase form a protein complex in peroxisomes [16].
  • Immunoprecipitation experiments showed complete precipitation of DHAPAT activity in fractions prepared from human placenta, liver and skin fibroblasts [17].
  • The peroxisomal enzyme acyl-CoA:dihydroxyacetonephosphate acyltransferase (DHAPAT) was extracted from human placental membranes using CHAPS as a detergent in the presence of 1 M KCl [17].
  • Both the DHAP acyltransferase (DHAP-AT) and alkyl-DHAP synthase activities were located inside glycosomes whereas the alkyl/acyl-DHAP oxidoreductase activity was associated with the cytoplasmic face of the glycosomal membrane [18].
 

Associations of GNPAT with chemical compounds

  • Deficient de novo plasmalogen synthesis in her fibroblasts as a result of low DHAPAT activity was found, while her very-long-chain fatty acid profile, phytanic acid concentration, alkyl-dihydroxyacetonephosphate synthase (alkyl-DHAP synthase) activity, and peroxisomal 3-ketoacyl-CoA thiolase protein were normal [1].
  • A mutation in her DHAPAT complementary DNA resulted in the substitution of an arginine residue in the protein at position 211 by a histidine (R211H) [1].
  • To enable these studies, the usually employed assay conditions for DHAP-AT were modified by omission of BSA and by lowering the temperature and the palmitoyl-CoA concentration [19].
  • Biochemical abnormalities including defective lignoceric acid oxidation, dihydroxyacetone phosphate acyltransferase deficiency, and disturbed biosynthesis of peroxisomal beta-oxidation enzymes were preserved in the transformants [20].
  • A fairly good correlation was found for pristanic acid beta-oxidation, but the best correlation was found for DHAPAT activity and C26:0 beta-oxidation [12].
 

Regulatory relationships of GNPAT

 

Other interactions of GNPAT

 

Analytical, diagnostic and therapeutic context of GNPAT

References

  1. Abnormal myelin formation in rhizomelic chondrodysplasia punctata type 2 (DHAPAT-deficiency). Sztriha, L., Al-Gazali, L.I., Wanders, R.J., Ofman, R., Nork, M., Lestringant, G.G. Developmental medicine and child neurology. (2000) [Pubmed]
  2. HMG-CoA reductase inhibitors perturb fatty acid metabolism and induce peroxisomes in keratinocytes. Williams, M.L., Menon, G.K., Hanley, K.P. J. Lipid Res. (1992) [Pubmed]
  3. Lipid composition of hepatitis B virus surface antigen particles and the particle-producing human hepatoma cell lines. Satoh, O., Umeda, M., Imai, H., Tunoo, H., Inoue, K. J. Lipid Res. (1990) [Pubmed]
  4. Congenital rubella syndrome associated with calcific epiphyseal stippling and peroxisomal dysfunction. Pike, M.G., Applegarth, D.A., Dunn, H.G., Bamforth, S.J., Tingle, A.J., Wood, B.J., Dimmick, J.E., Harris, H., Chantler, J.K., Hall, J.G. J. Pediatr. (1990) [Pubmed]
  5. Deficiency of acyl-CoA: dihydroxyacetone phosphate acyltransferase in patients with Zellweger (cerebro-hepato-renal) syndrome. Schutgens, R.B., Romeyn, G.J., Wanders, R.J., van den Bosch, H., Schrakamp, G., Heymans, H.S. Biochem. Biophys. Res. Commun. (1984) [Pubmed]
  6. Acyl-CoA:dihydroxyacetonephosphate acyltransferase: cloning of the human cDNA and resolution of the molecular basis in rhizomelic chondrodysplasia punctata type 2. Ofman, R., Hettema, E.H., Hogenhout, E.M., Caruso, U., Muijsers, A.O., Wanders, R.J. Hum. Mol. Genet. (1998) [Pubmed]
  7. Leishmania major expresses a single dihydroxyacetone phosphate acyltransferase localized in the glycosome, important for rapid growth and survival at high cell density and essential for virulence. Zufferey, R., Ben Mamoun, C. J. Biol. Chem. (2006) [Pubmed]
  8. A Single Nucleotide Polymorphism Fine Mapping Study of Chromosome 1q42.1 Reveals the Vulnerability Genes for Schizophrenia, GNPAT and DISC1: Association with Impairment of Sustained Attention. Liu, Y.L., Fann, C.S., Liu, C.M., Chen, W.J., Wu, J.Y., Hung, S.I., Chen, C.H., Jou, Y.S., Liu, S.K., Hwang, T.J., Hsieh, M.H., Ouyang, W.C., Chan, H.Y., Chen, J.J., Yang, W.C., Lin, C.Y., Lee, S.F., Hwu, H.G. Biol. Psychiatry (2006) [Pubmed]
  9. Effect of hypoxia-reoxygenation on peroxisomal functions in cultured human skin fibroblasts from control and Zellweger syndrome patients. Kremser, K., Kremser-Jezik, M., Singh, I. Free Radic. Res. (1995) [Pubmed]
  10. The cerebro-hepato-renal (Zellweger) syndrome: prenatal detection based on impaired biosynthesis of plasmalogens. Schutgens, R.B., Schrakamp, G., Wanders, R.J., Heymans, H.S., Moser, H.W., Moser, A.E., Tager, J.M., Bosch, H.V., Aubourg, P. Prenat. Diagn. (1985) [Pubmed]
  11. Role of dihydroxyacetonephosphate acyltransferase in the biosynthesis of plasmalogens and nonether glycerolipids. Liu, D., Nagan, N., Just, W.W., Rodemer, C., Thai, T.P., Zoeller, R.A. J. Lipid Res. (2005) [Pubmed]
  12. Biochemical markers predicting survival in peroxisome biogenesis disorders. Gootjes, J., Mooijer, P.A., Dekker, C., Barth, P.G., Poll-The, B.T., Waterham, H.R., Wanders, R.J. Neurology (2002) [Pubmed]
  13. Novel mutations in the PEX12 gene of patients with a peroxisome biogenesis disorder. Gootjes, J., Schmohl, F., Waterham, H.R., Wanders, R.J. Eur. J. Hum. Genet. (2004) [Pubmed]
  14. Prenatal diagnosis of rhizomelic chondrodysplasia punctata due to isolated alkyldihydroacetonephosphate acyltransferase synthase deficiency. Brookhyser, K.M., Lipson, M.H., Moser, A.B., Moser, H.W., Lachman, R.S., Rimoin, D.L. Prenat. Diagn. (1999) [Pubmed]
  15. Measurement of dihydroxyacetone-phosphate acyltransferase (DHAPAT) in chorionic villous samples, blood cells and cultured cells. Wanders, R.J., Ofman, R., Romeijn, G.J., Schutgens, R.B., Mooijer, P.A., Dekker, C., van den Bosch, H. J. Inherit. Metab. Dis. (1995) [Pubmed]
  16. Alkyl-dihydroxyacetone phosphate synthase and dihydroxyacetone phosphate acyltransferase form a protein complex in peroxisomes. Biermann, J., Just, W.W., Wanders, R.J., Van Den Bosch, H. Eur. J. Biochem. (1999) [Pubmed]
  17. Purification of peroxisomal acyl-CoA: dihydroxyacetonephosphate acyltransferase from human placenta. Ofman, R., Wanders, R.J. Biochim. Biophys. Acta (1994) [Pubmed]
  18. The dihydroxyacetonephosphate pathway for biosynthesis of ether lipids in Leishmania mexicana promastigotes. Heise, N., Opperdoes, F.R. Mol. Biochem. Parasitol. (1997) [Pubmed]
  19. Topography of ether phospholipid biosynthesis. Hardeman, D., van den Bosch, H. Biochim. Biophys. Acta (1989) [Pubmed]
  20. Transformation and characterization of mutant human fibroblasts defective in peroxisome assembly. Okamoto, H., Suzuki, Y., Shimozawa, N., Yajima, S., Masuno, M., Orii, T. Exp. Cell Res. (1992) [Pubmed]
  21. Properties of the enzymes catalyzing the biosynthesis of lysophosphatidate and its ether analog in cultured fibroblasts from Zellweger syndrome patients and normal controls. Webber, K.O., Datta, N.S., Hajra, A.K. Arch. Biochem. Biophys. (1987) [Pubmed]
  22. X-linked dominant chondrodysplasia punctata with decreased dihydroxyacetone phosphate acyltransferase activity. Sato, M., Ishikawa, O., Miyachi, Y. Dermatology (Basel) (1996) [Pubmed]
  23. Peroxisomal function is altered during leishmania infection. Raychaudhury, B., Banerjee, S., Datta, S.C. Med. Sci. Monit. (2003) [Pubmed]
  24. Plasmalogens in the retina: In situ hybridization of dihydroxyacetone phosphate acyltransferase (DHAP-AT) - the first enzyme involved in their biosynthesis - and comparative study of retinal and retinal pigment epithelial lipid composition. Acar, N., Gregoire, S., Andre, A., Juaneda, P., Joffre, C., Bron, A.M., Creuzot-Garcher, C.P., Bretillon, L. Exp. Eye Res. (2007) [Pubmed]
 
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