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Gene Review

Cps1  -  carbamoyl-phosphate synthetase 1

Mus musculus

Synonyms: 4732433M03Rik, CPS, CPSase I, Carbamoyl-phosphate synthetase I, D1Ucla3
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Disease relevance of Cps1


High impact information on Cps1


Chemical compound and disease context of Cps1


Biological context of Cps1


Anatomical context of Cps1


Associations of Cps1 with chemical compounds

  • Furthermore, there was an increase in the hepatic ornithine-urea cycle (OUC) capacity, with significant increases in the activities of CPS III (3.8-fold), argininosuccinate synthetase + lyase (1.8-fold) and, more importantly, glutamine synthetase (2.2-fold) [18].
  • Like the marine ray Taeniura lymma, the African lungfish Protopterus dolloi possesses carbamoyl phosphate III (CPS III) in the liver and not carbamoyl phosphate I (CPS I), as in the mouse Mus musculus or as in other African lungfish reported elsewhere [18].
  • Carnitine administration relieved the suppression of the developmental induction of two urea cycle enzymes examined, carbamoyl-phosphate synthetase and argininosuccinate synthase, and kept the activities of enzymes normal [19].
  • Addition of a synthetic glucocorticoid hormone, dexamethasone, caused increases in CPS and ASS mRNAs [13].
  • Further addition of oleic acid suppressed the induction of CPS and ASS mRNAs by dexamethasone [13].

Other interactions of Cps1


Analytical, diagnostic and therapeutic context of Cps1

  • The levels and also the authenticity of translation of rat liver albumin and mouse liver carbamoyl phosphate synthetase I were tested by immunoprecipitation with monospecific antibodies [23].
  • Specific antibody was used to stabilize the CPS on the surface of K. pneumoniae, and the structural organization of the homologous and heterologous capsules was examined by electron microscopy [3].
  • This ELISA allowed selection of an appropriate vaccine strain, for a natural polysaccharide vaccine, among several 23F pneumococcal clinical isolates and constituted a valuable analytical tool for Strep. pneumoniae fermentation and CPS purification follow-up [11].
  • N-Acetyl-L-glutamate (NAG), the activator of mitochondrial carbamoyl phosphate synthetase (CPS), is demonstrated by several methods, including a new HPLC assay, in the brain of mammals and of chicken [24].


  1. Mice deficient in the urea-cycle enzyme, carbamoyl phosphate synthetase I, die during the early neonatal period from hyperammonemia. Schofield, J.P., Cox, T.M., Caskey, C.T., Wakamiya, M. Hepatology (1999) [Pubmed]
  2. Hepatic carbamyl phosphate synthetase and ornithine transcarbamylase in mouse influenze A and influenze B infection. Pierson, D., Knight, V., Hansard, P., Chan, E. Proc. Soc. Exp. Biol. Med. (1976) [Pubmed]
  3. Genetic exchange of determinants for capsular polysaccharide biosynthesis between Klebsiella pneumoniae strains expressing serotypes K2 and K21a. Ofek, I., Kabha, K., Athamna, A., Frankel, G., Wozniak, D.J., Hasty, D.L., Ohman, D.E. Infect. Immun. (1993) [Pubmed]
  4. Posttranscriptional regulation of the expression of CAD gene during differentiation of F9 teratocarcinoma cells by induction with retinoic acid and dibutyryl cyclic AMP. Rao, G.N., Church, R.L., Davidson, J.N. FEBS Lett. (1988) [Pubmed]
  5. Metabolism of chlorpyrifos by human cytochrome P450 isoforms and human, mouse, and rat liver microsomes. Tang, J., Cao, Y., Rose, R.L., Brimfield, A.A., Dai, D., Goldstein, J.A., Hodgson, E. Drug Metab. Dispos. (2001) [Pubmed]
  6. De novo pyrimidine biosynthesis is required for virulence of Toxoplasma gondii. Fox, B.A., Bzik, D.J. Nature (2002) [Pubmed]
  7. Glucocorticoid receptor, C/EBP, HNF3, and protein kinase A coordinately activate the glucocorticoid response unit of the carbamoylphosphate synthetase I gene. Christoffels, V.M., Grange, T., Kaestner, K.H., Cole, T.J., Darlington, G.J., Croniger, C.M., Lamers, W.H. Mol. Cell. Biol. (1998) [Pubmed]
  8. The upstream regulatory region of the carbamoyl-phosphate synthetase I gene controls its tissue-specific, developmental, and hormonal regulation in vivo. Christoffels, V.M., van den Hoff, M.J., Lamers, M.C., van Roon, M.A., de Boer, P.A., Moorman, A.F., Lamers, W.H. J. Biol. Chem. (1996) [Pubmed]
  9. A mitochondrial protease that cleaves the precursor of ornithine carbamoyltransferase. Purification and properties. Miura, S., Mori, M., Amaya, Y., Tatibana, M. Eur. J. Biochem. (1982) [Pubmed]
  10. Effects of acivicin and PALA, singly and in combination, on de novo pyrimidine biosynthesis. Kensler, T.W., Jayaram, H.N., Cooney, D.A. Adv. Enzyme Regul. (1982) [Pubmed]
  11. Enzyme-linked immunosorbent assay for quantitative determination of capsular polysaccharide production in Streptococcus pneumoniae clinical isolates. Cruz-Leal, Y., Menéndez, T., Coizeau, E., Espinosa, R.R., Canaan, L., Blanco, F., Carmenate, T., Chang, J., Quiñones, D., Tamargo, I., Cremata, J., Verez-Bencomo, V., Guillén, G. Biotechnol. Appl. Biochem. (2006) [Pubmed]
  12. Paraoxonase protects against chlorpyrifos toxicity in mice. Li, W.F., Furlong, C.E., Costa, L.G. Toxicol. Lett. (1995) [Pubmed]
  13. Long-chain fatty acids suppress the induction of urea cycle enzyme genes by glucocorticoid action. Tomomura, M., Tomomura, A., Dewan, M.A., Saheki, T. FEBS Lett. (1996) [Pubmed]
  14. Caspase-dependent cleavage of carbamoyl phosphate synthetase II during apoptosis. Huang, M., Kozlowski, P., Collins, M., Wang, Y., Haystead, T.A., Graves, L.M. Mol. Pharmacol. (2002) [Pubmed]
  15. Group B Streptococcus capsular polysaccharide-cholera toxin B subunit conjugate vaccines prepared by different methods for intranasal immunization. Shen, X., Lagergård, T., Yang, Y., Lindblad, M., Fredriksson, M., Holmgren, J. Infect. Immun. (2001) [Pubmed]
  16. Ornithine-urea cycle and urea synthesis in African lungfishes, Protopterus aethiopicus and Protopterus annectens, exposed to terrestrial conditions for six days. Loong, A.M., Hiong, K.C., Lee, S.M., Wong, W.P., Chew, S.F., Ip, Y.K. J. Exp. Zoolog. Part A Comp. Exp. Biol. (2005) [Pubmed]
  17. Developmental appearance of ammonia-metabolizing enzymes in prenatal murine liver. Notenboom, R.G., Moorman, A.F., Lamers, W.H. Microsc. Res. Tech. (1997) [Pubmed]
  18. Urea synthesis in the African lungfish Protopterus dolloi--hepatic carbamoyl phosphate synthetase III and glutamine synthetase are upregulated by 6 days of aerial exposure. Chew, S.F., Ong, T.F., Ho, L., Tam, W.L., Loong, A.M., Hiong, K.C., Wong, W.P., Ip, Y.K. J. Exp. Biol. (2003) [Pubmed]
  19. Carnitine administration to juvenile visceral steatosis mice corrects the suppressed expression of urea cycle enzymes by normalizing their transcription. Horiuchi, M., Kobayashi, K., Tomomura, M., Kuwajima, M., Imamura, Y., Koizumi, T., Nikaido, H., Hayakawa, J., Saheki, T. J. Biol. Chem. (1992) [Pubmed]
  20. Expression of three mitochondrial solute carriers, citrin, aralar1 and ornithine transporter, in relation to urea cycle in mice. Begum, L., Jalil, M.A., Kobayashi, K., Iijima, M., Li, M.X., Yasuda, T., Horiuchi, M., del Arco, A., Satrústegui, J., Saheki, T. Biochim. Biophys. Acta (2002) [Pubmed]
  21. Suppressed expression of the urea cycle enzyme genes in the liver of carnitine-deficient juvenile visceral steatosis (JVS) mice in infancy and during starvation in adulthood. Tomomura, M., Tomomura, A., Musa, D.A., Horiuchi, M., Takiguchi, M., Mori, M., Saheki, T. J. Biochem. (1997) [Pubmed]
  22. Long-term culture and differentiation of rat embryonic stem cell-like cells into neuronal, glial, endothelial, and hepatic lineages. Ruhnke, M., Ungefroren, H., Zehle, G., Bader, M., Kremer, B., Fändrich, F. Stem Cells (2003) [Pubmed]
  23. Polysome-dependent in vitro translation system capable of peptide chain reinitiation. Aroskar, V.A., Watt, R.A., Emeh, J.K., Niranjan, B.G., Biunno, I., Avadhani, N.G. Biochemistry (1980) [Pubmed]
  24. N-acetyl-L-glutamate in brain: assay, levels, and regional and subcellular distribution. Alonso, E., García-Pérez, M.A., Bueso, J., Rubio, V. Neurochem. Res. (1991) [Pubmed]
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