The world's first wiki where authorship really matters (Nature Genetics, 2008). Due credit and reputation for authors. Imagine a global collaborative knowledge base for original thoughts. Search thousands of articles and collaborate with scientists around the globe.

wikigene or wiki gene protein drug chemical gene disease author authorship tracking collaborative publishing evolutionary knowledge reputation system wiki2.0 global collaboration genes proteins drugs chemicals diseases compound
Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)



Gene Review

Otc  -  ornithine carbamoyltransferase

Rattus norvegicus

Synonyms: OTCase, Ornithine carbamoyltransferase, mitochondrial, Ornithine transcarbamylase
Welcome! If you are familiar with the subject of this article, you can contribute to this open access knowledge base by deleting incorrect information, restructuring or completely rewriting any text. Read more.

Disease relevance of Otc


High impact information on Otc

  • Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide [5].
  • Substitution of either an acidic (aspartate) or a "helix-breaking" (glycine) amino acid residue for arginine 23 of the leader inhibits formation of both iOTC and OTC, without affecting translocation [5].
  • The mitochondrial matrix enzyme ornithine transcarbamylase (OTC) is synthesized on cytoplasmic polyribosomes as a precursor (pOTC) with an NH2-terminal extension of 32 amino acids [5].
  • The same intermediate-sized OTC is produced in vivo in frog oocytes injected with in vitro-synthesized pOTC [5].
  • In the first step, which is dependent upon an intact mitochondrial membrane potential, pOTC is translocated into mitochondria and cleaved by a matrix protease to a product designated iOTC, intermediate in size between pOTC and mature OTC [5].

Chemical compound and disease context of Otc


Biological context of Otc


Anatomical context of Otc


Associations of Otc with chemical compounds


Other interactions of Otc


Analytical, diagnostic and therapeutic context of Otc


  1. Selection of a cDNA clone which contains the complete coding sequence for the mature form of ornithine transcarbamylase from rat liver: expression of the cloned protein in Escherichia coli. Molecular cloning of rat ornithine transcarbamylase. McIntyre, P., Mercer, J.F., Peterson, M.G., Hudson, P., Hoogenraad, N. Eur. J. Biochem. (1984) [Pubmed]
  2. Promoter and 11-kilobase upstream enhancer elements responsible for hepatoma cell-specific expression of the rat ornithine transcarbamylase gene. Murakami, T., Nishiyori, A., Takiguchi, M., Mori, M. Mol. Cell. Biol. (1990) [Pubmed]
  3. Zinc supplementation reduces blood ammonia and increases liver ornithine transcarbamylase activity in experimental cirrhosis. Riggio, O., Merli, M., Capocaccia, L., Caschera, M., Zullo, A., Pinto, G., Gaudio, E., Franchitto, A., Spagnoli, R., D'Aquilino, E. Hepatology (1992) [Pubmed]
  4. Hepatic glutamine metabolism in the septic rat. Salleh, M., Ardawi, M. Clin. Sci. (1992) [Pubmed]
  5. Import of rat ornithine transcarbamylase precursor into mitochondria: two-step processing of the leader peptide. Sztul, E.S., Hendrick, J.P., Kraus, J.P., Wall, D., Kalousek, F., Rosenberg, L.E. J. Cell Biol. (1987) [Pubmed]
  6. Effects of zinc on hepatic ornithine transcarbamylase (OTC) activity. Aquilio, E., Spagnoli, R., Riggio, D., Seri, S. Journal of trace elements and electrolytes in health and disease. (1993) [Pubmed]
  7. A cDNA clone for the precursor of rat mitochondrial ornithine transcarbamylase: comparison of rat and human leader sequences and conservation of catalytic sites. Kraus, J.P., Hodges, P.E., Williamson, C.L., Horwich, A.L., Kalousek, F., Williams, K.R., Rosenberg, L.E. Nucleic Acids Res. (1985) [Pubmed]
  8. A highly basic N-terminal extension of the mitochondrial matrix enzyme ornithine transcarbamylase from rat liver. McIntyre, P., Graf, L., Mercer, J., Peterson, G., Hudson, P., Hoogenraad, N. FEBS Lett. (1984) [Pubmed]
  9. The primary structure of the imported mitochondrial protein, ornithine transcarbamylase from rat liver: mRNA levels during ontogeny. McIntyre, P., Graf, L., Mercer, J.F., Wake, S.A., Hudson, P., Hoogenraad, N. DNA (1985) [Pubmed]
  10. Newly processed ornithine transcarbamylase subunits are assembled to trimers in rat liver mitochondria. Kalousek, F., Orsulak, M.D., Rosenberg, L.E. J. Biol. Chem. (1984) [Pubmed]
  11. Biotin deficiency decreases ornithine transcarbamylase activity and mRNA in rat liver. Maeda, Y., Kawata, S., Inui, Y., Fukuda, K., Igura, T., Matsuzawa, Y. J. Nutr. (1996) [Pubmed]
  12. Characterization of a protease apparently involved in processing of pre-ornithine transcarbamylase of rat liver. Mori, M., Miura, S., Tatibana, M., Cohen, P.P. Proc. Natl. Acad. Sci. U.S.A. (1980) [Pubmed]
  13. A noncleavable signal for mitochondrial import of 3-oxoacyl-CoA thiolase. Amaya, Y., Arakawa, H., Takiguchi, M., Ebina, Y., Yokota, S., Mori, M. J. Biol. Chem. (1988) [Pubmed]
  14. Transfection of mitochondria: strategy towards a gene therapy of mitochondrial DNA diseases. Seibel, P., Trappe, J., Villani, G., Klopstock, T., Papa, S., Reichmann, H. Nucleic Acids Res. (1995) [Pubmed]
  15. Site-directed mutagenesis of Arg60 and Cys271 in ornithine transcarbamylase from rat liver. McDowall, S., van Heeswijck, R., Hoogenraad, N. Protein Eng. (1990) [Pubmed]
  16. Synthesis, intracellular transport, and processing of the precursors for mitochondrial ornithine transcarbamylase and carbamoyl-phosphate synthetase I in isolated hepatocytes. Mori, M., Morita, T., Ikeda, F., Amaya, Y., Tatibana, M., Cohen, P.P. Proc. Natl. Acad. Sci. U.S.A. (1981) [Pubmed]
  17. Mitochondrial carbamyl phosphate and citrulline synthesis at high matrix acetylglutamate. Cohen, N.S., Cheung, C.W., Kyan, F.S., Jones, E.E., Raijman, L. J. Biol. Chem. (1982) [Pubmed]
  18. Channeling of urea cycle intermediates in situ in permeabilized hepatocytes. Cheung, C.W., Cohen, N.S., Raijman, L. J. Biol. Chem. (1989) [Pubmed]
  19. Uptake and processing of the precursor for rat liver ornithine transcarbamylase by isolated mitochondria. Inhibition by uncouplers. Mori, M., Morita, T., Miura, S., Tatibana, M. J. Biol. Chem. (1981) [Pubmed]
  20. Hemin administration to rats reduces levels of hepatic mRNAs for phenobarbitone-inducible enzymes. Srivastava, G., Hansen, A.J., Bawden, M.J., May, B.K. Mol. Pharmacol. (1990) [Pubmed]
  21. Adaptation in enzyme (metabolic) pathways to obesity, carbohydrate diet and to the occurrence of NIDDM in male and female SHR/N-cp rats. Yamini, S., Carswell, N., Michaelis, O.E., Szepesi, B. Int. J. Obes. Relat. Metab. Disord. (1992) [Pubmed]
  22. Turnover of rat liver ornithine transcarbamylase. Wallace, R., Knecht, E., Grisolía, S. FEBS Lett. (1986) [Pubmed]
  23. Complementation of the Aspergillus nidulans arg B1 mutation by ornithine transcarbamylase cDNA from rat liver. van Heeswijck, R., Ristevski, S., Hynes, M., Hoogenraad, N. Biochem. Biophys. Res. Commun. (1990) [Pubmed]
  24. Immunocytochemical localization of ornithine transcarbamylase in rat intestinal mucosa. Light and electron microscopic study. Hamano, Y., Kodama, H., Yanagisawa, M., Haraguchi, Y., Mori, M., Yokota, S. J. Histochem. Cytochem. (1988) [Pubmed]
WikiGenes - Universities