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Chemical Compound Review

ornithine     (2R)-2,5-diaminopentanoic acid

Synonyms: d-ornithin, D-ornithine, AmbotzHAA1041, CHEMBL103686, AG-F-19709, ...
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Disease relevance of ornithine


Psychiatry related information on ornithine


High impact information on ornithine


Chemical compound and disease context of ornithine


Biological context of ornithine


Anatomical context of ornithine


Associations of ornithine with other chemical compounds


Gene context of ornithine

  • Moreover, activation of p42/p44 and induction of p21(waf1/cip1) were prevented by exogenous putrescine but not ornithine, suggesting this effect was due to the inhibition of ODC by NO or DFMO [31].
  • Importantly, arginase synthesizes ornithine, which is metabolized by the enzyme ornithine decarboxylase (ODC) to produce polyamines [32].
  • In OTCase, mutations of putative ornithine binding residues, Asp-182, Asn-184, Asn-185, Cys-289, and Glu-256 greatly reduced the affinity for ornithine and impaired the interaction with arginase [33].
  • Expression of either I(1,4,5)P3 6- or 3-kinase activity rescued growth of ipk2-deficient yeast at high temperatures, whereas only 6-kinase activity enabled growth on ornithine as the sole nitrogen source [34].
  • The product of the bioA gene, 7,8-diaminopelargonic acid aminotransferase, was discovered to be related to ornithine aminotransferase [35].

Analytical, diagnostic and therapeutic context of ornithine


  1. Evolutionary drift of the argF and argl genes. Coding for isoenzyme forms of ornithine transcarbamylase in E. coli K12. Sens, D., Natter, W., James, E. Cell (1977) [Pubmed]
  2. Gyrate atrophy of choroid and retina: deficient activity of ornithine ketoacid aminotransferase in cultured skin fibroblasts. Kennaway, N.G., Weleber, R.G., Buist, N.R. N. Engl. J. Med. (1977) [Pubmed]
  3. Letter: Ornithine or citrulline therapy in treatment of Reye's syndrome. Tremblay, G.C. N. Engl. J. Med. (1975) [Pubmed]
  4. Mice lacking ornithine-delta-aminotransferase have paradoxical neonatal hypoornithinaemia and retinal degeneration. Wang, T., Lawler, A.M., Steel, G., Sipila, I., Milam, A.H., Valle, D. Nat. Genet. (1995) [Pubmed]
  5. Increased ornithine decarboxylase activity in murine sarcoma virus infected cells. Gazdar, A.F., Stull, H.B., Kilton, L.J., Bachrach, U. Nature (1976) [Pubmed]
  6. Ornithine aminotransferase in Huntington's disease. Wong, P.T., McGeer, P.L., Rossor, M., McGeer, E.G. Brain Res. (1982) [Pubmed]
  7. Amino acid concentrations in cerebrospinal fluid in presenile and senile dementia of Alzheimer type and multi-infarct dementia. Degrell, I., Hellsing, K., Nagy, E., Niklasson, F. Archives of gerontology and geriatrics. (1989) [Pubmed]
  8. The hyperornithinemia, hyperammonemia, homocitrullinuria syndrome: an ornithine transport defect remediable with ornithine supplements. Gordon, B.A., Gatfield, D.P., Haust, M.D. Clinical and investigative medicine. Médecine clinique et experimentale. (1987) [Pubmed]
  9. Hyperornithinaemia-hyperammonaemia-homocitrullinuria syndrome is caused by mutations in a gene encoding a mitochondrial ornithine transporter. Camacho, J.A., Obie, C., Biery, B., Goodman, B.K., Hu, C.A., Almashanu, S., Steel, G., Casey, R., Lambert, M., Mitchell, G.A., Valle, D. Nat. Genet. (1999) [Pubmed]
  10. Supplementary creatine as a treatment for gyrate atrophy of the choroid and retina. Sipilä, I., Rapola, J., Simell, O., Vannas, A. N. Engl. J. Med. (1981) [Pubmed]
  11. Transiently reduced activity of carbamyl phosphate synthetase and ornithine transcarbamylase in liver of children with Reye's syndrome. Brown, T., Hug, G., Lansky, L., Bove, K., Scheve, A., Ryan, M., Brown, H., Schubert, W.K., Partin, J.C., Lloyd-Still, J. N. Engl. J. Med. (1976) [Pubmed]
  12. Reconstruction of an enzyme by domain substitution effectively switches substrate specificity. Houghton, J.E., O'Donovan, G.A., Wild, J.R. Nature (1989) [Pubmed]
  13. Gyrate atrophy of the choroid and retina: improved visual function following reduction of plasma ornithine by diet. Kaiser-Kupfer, M.I., de Monasterio, F.M., Valle, D., Walser, M., Brusilow, S. Science (1980) [Pubmed]
  14. Renal ammoniagenesis in an early stage of metabolic acidosis in man. Tizianello, A., Deferrari, G., Garibotto, G., Robaudo, C., Acquarone, N., Ghiggeri, G.M. J. Clin. Invest. (1982) [Pubmed]
  15. Masking by enzyme inhibitor of raised serum glutamate dehydrogenase activity in Reye's syndrome. Holt, J.T., Arvan, D.A., Mayer, T.K. Lancet (1983) [Pubmed]
  16. Liver transplantation improves hepatic myelopathy: evidence by three cases. Weissenborn, K., Tietge, U.J., Bokemeyer, M., Mohammadi, B., Bode, U., Manns, M.P., Caselitz, M. Gastroenterology (2003) [Pubmed]
  17. Ornithine decarboxylase: essential in proliferation but not differentiation of human promyelocytic leukemia cells. Luk, G.D., Civin, C.I., Weissman, R.M., Baylin, S.B. Science (1982) [Pubmed]
  18. Triggering of allostery in an enzyme by a point mutation: ornithine transcarbamoylase. Kuo, L.C., Zambidis, I., Caron, C. Science (1989) [Pubmed]
  19. Gyrate atrophy of the choroid and retina with hyperornithinemia: characterization of mutant liver L-ornithine:2-oxoacid aminotransferase kinetics. Sipilä, I., Simell, O., O'Donnell, J.J. J. Clin. Invest. (1981) [Pubmed]
  20. A mouse model of gyrate atrophy of the choroid and retina. Early retinal pigment epithelium damage and progressive retinal degeneration. Wang, T., Milam, A.H., Steel, G., Valle, D. J. Clin. Invest. (1996) [Pubmed]
  21. Phosphorylation of ornithine decarboxylase by a polyamine-dependent protein kinase. Atmar, V.J., Kuehn, G.D. Proc. Natl. Acad. Sci. U.S.A. (1981) [Pubmed]
  22. Gyrate atrophy of the choroid and retina with hyperornithinemia. Deficient formation of guanidinoacetic acid from arginine. Sipilä, I., Simell, O., Arjomaa, P. J. Clin. Invest. (1980) [Pubmed]
  23. Putrescine is involved in the vitamin D action in chick intestine. Shinki, T., Tanaka, H., Takito, J., Yamaguchi, A., Nakamura, Y., Yoshiki, S., Suda, T. Gastroenterology (1991) [Pubmed]
  24. Import and processing of human ornithine transcarbamoylase precursor by mitochondria from Saccharomyces cerevisiae. Cheng, M.Y., Pollock, R.A., Hendrick, J.P., Horwich, A.L. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  25. Posttranslational uptake and processing of in vitro synthesized ornithine transcarbamoylase precursor by isolated rat liver mitochondria. Conboy, J.G., Rosenberg, L.E. Proc. Natl. Acad. Sci. U.S.A. (1981) [Pubmed]
  26. Urea-cycle enzyme deficiencies and an increased nitrogen load producing hyperammonemia in Reye's syndrome. Snodgrass, P.J., DeLong, G.R. N. Engl. J. Med. (1976) [Pubmed]
  27. Polyamine metabolism: a potential therapeutic target in trypanosomes. Bacchi, C.J., Nathan, H.C., Hutner, S.H., McCann, P.P., Sjoerdsma, A. Science (1980) [Pubmed]
  28. Role of epidermal growth factor, prostaglandin, and sulfhydryls in stress-induced gastric lesions. Konturek, P.K., Brzozowski, T., Konturek, S.J., Dembiński, A. Gastroenterology (1990) [Pubmed]
  29. Regulation of polyamine biosynthesis in Escherichia coli by basic proteins. Heller, J.S., Rostomily, R., Kyriakidis, D.A., Canellakis, E.S. Proc. Natl. Acad. Sci. U.S.A. (1983) [Pubmed]
  30. Structure of the rat ornithine carbamoyltransferase gene, a large, X chromosome-linked gene with an atypical promoter. Takiguchi, M., Murakami, T., Miura, S., Mori, M. Proc. Natl. Acad. Sci. U.S.A. (1987) [Pubmed]
  31. Role of p42/p44 mitogen-activated-protein kinase and p21waf1/cip1 in the regulation of vascular smooth muscle cell proliferation by nitric oxide. Bauer, P.M., Buga, G.M., Ignarro, L.J. Proc. Natl. Acad. Sci. U.S.A. (2001) [Pubmed]
  32. Protective role of arginase in a mouse model of colitis. Gobert, A.P., Cheng, Y., Akhtar, M., Mersey, B.D., Blumberg, D.R., Cross, R.K., Chaturvedi, R., Drachenberg, C.B., Boucher, J.L., Hacker, A., Casero, R.A., Wilson, K.T. J. Immunol. (2004) [Pubmed]
  33. Yeast epiarginase regulation, an enzyme-enzyme activity control: identification of residues of ornithine carbamoyltransferase and arginase responsible for enzyme catalytic and regulatory activities. El Alami, M., Dubois, E., Oudjama, Y., Tricot, C., Wouters, J., Stalon, V., Messenguy, F. J. Biol. Chem. (2003) [Pubmed]
  34. Molecular definition of a novel inositol polyphosphate metabolic pathway initiated by inositol 1,4,5-trisphosphate 3-kinase activity in Saccharomyces cerevisiae. Seeds, A.M., Bastidas, R.J., York, J.D. J. Biol. Chem. (2005) [Pubmed]
  35. The Escherichia coli biotin biosynthetic enzyme sequences predicted from the nucleotide sequence of the bio operon. Otsuka, A.J., Buoncristiani, M.R., Howard, P.K., Flamm, J., Johnson, C., Yamamoto, R., Uchida, K., Cook, C., Ruppert, J., Matsuzaki, J. J. Biol. Chem. (1988) [Pubmed]
  36. Tumor suppression with a combination of alpha-difluoromethyl ornithine and interferon. Sunkara, P.S., Prakash, N.J., Mayer, G.D., Sjoerdsma, A. Science (1983) [Pubmed]
  37. Fetal liver biopsy for prenatal diagnosis of ornithine carbamyl transferase deficiency. Rodeck, C.H., Patrick, A.D., Pembrey, M.E., Tzannatos, C., Whitfield, A.E. Lancet (1982) [Pubmed]
  38. Molecular cloning of human ornithine aminotransferase mRNA. Inana, G., Totsuka, S., Redmond, M., Dougherty, T., Nagle, J., Shiono, T., Ohura, T., Kominami, E., Katunuma, N. Proc. Natl. Acad. Sci. U.S.A. (1986) [Pubmed]
  39. Correction of ornithine accumulation prevents retinal degeneration in a mouse model of gyrate atrophy of the choroid and retina. Wang, T., Steel, G., Milam, A.H., Valle, D. Proc. Natl. Acad. Sci. U.S.A. (2000) [Pubmed]
  40. Blockade of the L-arginine/NO synthase pathway worsens hepatic apoptosis and liver transplant preservation injury. Yagnik, G.P., Takahashi, Y., Tsoulfas, G., Reid, K., Murase, N., Geller, D.A. Hepatology (2002) [Pubmed]
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