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Disease relevance of Hyperammonemia


Psychiatry related information on Hyperammonemia


High impact information on Hyperammonemia


Chemical compound and disease context of Hyperammonemia


Biological context of Hyperammonemia


Anatomical context of Hyperammonemia


Gene context of Hyperammonemia


Analytical, diagnostic and therapeutic context of Hyperammonemia


  1. Hyperinsulinism and hyperammonemia in infants with regulatory mutations of the glutamate dehydrogenase gene. Stanley, C.A., Lieu, Y.K., Hsu, B.Y., Burlina, A.B., Greenberg, C.R., Hopwood, N.J., Perlman, K., Rich, B.H., Zammarchi, E., Poncz, M. N. Engl. J. Med. (1998) [Pubmed]
  2. 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]
  3. Letter: Nonketotic hyperglycinemia with increased propionic acid excretion and hyperammonemia. Farriaux, J.P., Morel, P., Hommes, F.A. N. Engl. J. Med. (1976) [Pubmed]
  4. Ammonia intoxication in the near-adult cat as a result of a dietary deficiency of arginine. Morris, J.G., Rogers, Q.R. Science (1978) [Pubmed]
  5. Carnitine-acylcarnitine translocase deficiency with severe hypoglycemia and auriculo ventricular block. Translocase assay in permeabilized fibroblasts. Pande, S.V., Brivet, M., Slama, A., Demaugre, F., Aufrant, C., Saudubray, J.M. J. Clin. Invest. (1993) [Pubmed]
  6. Hyperammonemia secondary to valproic acid as a cause of lethargy in a postictal patient. Kulick, S.K., Kramer, D.A. Annals of emergency medicine. (1993) [Pubmed]
  7. The molecular basis of ornithine transcarbamylase deficiency. Tuchman, M., McCullough, B.A., Yudkoff, M. Eur. J. Pediatr. (2000) [Pubmed]
  8. The influence of NMDA, a potent agonist of glutamate receptor, on behavioral activity of rats with experimental hyperammonemia evoked by liver failure. Fedosiewicz-Wasiluk, M., Hoły, Z.Z., Wiśniewska, R.J., Wiśniewski, K. Amino Acids (2005) [Pubmed]
  9. Hyperammonemia and coma developed by a woman treated with valproic acid for affective disorder. Eze, E., Workman, M., Donley, B. Psychiatric services (Washington, D.C.) (1998) [Pubmed]
  10. Treatment of episodic hyperammonemia in children with inborn errors of urea synthesis. Brusilow, S.W., Danney, M., Waber, L.J., Batshaw, M., Burton, B., Levitsky, L., Roth, K., McKeethren, C., Ward, J. N. Engl. J. Med. (1984) [Pubmed]
  11. Ornithine transcarbamylase deficiency. Unsuccessful therapy of neonatal hyperammonemia with N-carbamyl-L-glutamate and L-arginine. Gelehrter, T.D., Rosenberg, L.E. N. Engl. J. Med. (1975) [Pubmed]
  12. Treatment of carbamyl phosphate synthetase deficiency with keto analogues of essential amino acids. Batshaw, M., Brusilow, S., Walser, M. N. Engl. J. Med. (1975) [Pubmed]
  13. Protective effect of tert-butanol on hyperammonemia. O'Connor, J.E., Guerri, C., Grisolía, S. N. Engl. J. Med. (1982) [Pubmed]
  14. Renal ammonia and glutamine metabolism during liver insufficiency-induced hyperammonemia in the rat. Dejong, C.H., Deutz, N.E., Soeters, P.B. J. Clin. Invest. (1993) [Pubmed]
  15. The association of hepatic glycogen depletion with hyperammonemia in cirrhosis. Kabadi, U.M. Hepatology (1987) [Pubmed]
  16. Novel intragenic deletions and point mutations of the ornithine transcarbamylase gene in congenital hyperammonemia. Calvas, P., Ségues, B., Rozet, J.M., Rabier, D., Bonnefond, J.P., Munnich, A. Hum. Mutat. (1998) [Pubmed]
  17. Valproic acid-induced hyperammonemia in mentally retarded adults. Williams, C.A., Tiefenbach, S., McReynolds, J.W. Neurology (1984) [Pubmed]
  18. Heterozygote ornithine transcarbamylase deficiency presenting as symptomatic hyperammonemia during initiation of valproate therapy. Honeycutt, D., Callahan, K., Rutledge, L., Evans, B. Neurology (1992) [Pubmed]
  19. Chronic exposure to ammonia alters pathways modulating phosphorylation of microtubule-associated protein 2 in cerebellar neurons in culture. Sáez, R., Llansola, M., Felipo, V. J. Neurochem. (1999) [Pubmed]
  20. Inhibition by propionyl-coenzyme A of N-acetylglutamate synthetase in rat liver mitochondria. A possible explanation for hyperammonemia in propionic and methylmalonic acidemia. Coude, F.X., Sweetman, L., Nyhan, W.L. J. Clin. Invest. (1979) [Pubmed]
  21. Effect of ammonium ions on synaptic transmission in the mammalian central nervous system. Szerb, J.C., Butterworth, R.F. Prog. Neurobiol. (1992) [Pubmed]
  22. Liver ultrastructure in mitochondrial urea cycle enzyme deficiencies and comparison with Reye's syndrome. Latham, P.S., LaBrecque, D.R., McReynolds, J.W., Klatskin, G. Hepatology (1984) [Pubmed]
  23. Changes in glutamate receptors on synaptic membranes associated with hepatic encephalopathy or hyperammonemia in the rabbit. Ferenci, P., Pappas, S.C., Munson, P.J., Jones, E.A. Hepatology (1984) [Pubmed]
  24. Cerebral cortex ammonia and glutamine metabolism in two rat models of chronic liver insufficiency-induced hyperammonemia: influence of pair-feeding. Dejong, C.H., Deutz, N.E., Soeters, P.B. J. Neurochem. (1993) [Pubmed]
  25. Unregulated elevation of glutamate dehydrogenase activity induces glutamine-stimulated insulin secretion: identification and characterization of a GLUD1 gene mutation and insulin secretion studies with MIN6 cells overexpressing the mutant glutamate dehydrogenase. Tanizawa, Y., Nakai, K., Sasaki, T., Anno, T., Ohta, Y., Inoue, H., Matsuo, K., Koga, M., Furukawa, S., Oka, Y. Diabetes (2002) [Pubmed]
  26. Genetic ablation of Rhbg in the mouse does not impair renal ammonium excretion. Chambrey, R., Goossens, D., Bourgeois, S., Picard, N., Bloch-Faure, M., Leviel, F., Geoffroy, V., Cambillau, M., Colin, Y., Paillard, M., Houillier, P., Cartron, J.P., Eladari, D. Am. J. Physiol. Renal Physiol. (2005) [Pubmed]
  27. Possible implication of ciliary neurotrophic factor (CNTF) and beta-synuclein in the ammonia effect on cultured rat astroglial cells: A study using DNA and protein microarrays. Bodega, G., Suárez, I., López-Fernández, L.A., Almonacid, L., Zaballos, A., Fernández, B. Neurochem. Int. (2006) [Pubmed]
  28. Activities of monoamine oxidase-A and -B are altered in the brains of congenitally hyperammonemic sparse-fur (spf) mice. Rao, V.L., Qureshi, I.A., Butterworth, R.F. Neurosci. Lett. (1994) [Pubmed]
  29. Seven novel mutations in the ORNT1 gene (SLC25A15) in patients with hyperornithinemia, hyperammonemia, and homocitrullinuria syndrome. Salvi, S., Dionisi-Vici, C., Bertini, E., Verardo, M., Santorelli, F.M. Hum. Mutat. (2001) [Pubmed]
  30. Hyperammonemic coma due to parenteral nutrition in a woman with heterozygous ornithine transcarbamylase deficiency. Felig, D.M., Brusilow, S.W., Boyer, J.L. Gastroenterology (1995) [Pubmed]
  31. In vivo 13C NMR measurements of cerebral glutamine synthesis as evidence for glutamate-glutamine cycling. Sibson, N.R., Dhankhar, A., Mason, G.F., Behar, K.L., Rothman, D.L., Shulman, R.G. Proc. Natl. Acad. Sci. U.S.A. (1997) [Pubmed]
  32. Oral administration of sildenafil restores learning ability in rats with hyperammonemia and with portacaval shunts. Erceg, S., Monfort, P., Hernández-Viadel, M., Rodrigo, R., Montoliu, C., Felipo, V. Hepatology (2005) [Pubmed]
  33. Quinolinate in brain and cerebrospinal fluid in rat models of congenital hyperammonemia. Robinson, M.B., Heyes, M.P., Anegawa, N.J., Gorry, E., Djali, S., Mellits, E.D., Batshaw, M.L. Pediatr. Res. (1992) [Pubmed]
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