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The Study of Organic Acids Metabolism in a Patient with Ornithine Transcarbamylase (OTC) Deficiency

  • Hiroko Kodama
  • Osamu Nose
  • Shintaro Okada
  • Hyakuji Yabuuchi
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

The formation of glutamate from ammonia and α-ketoglutarate (α-KG) is one of major pathways to remove excessive ammonia. This pathway is especially important in the urea cycle enzymopathies. It has been reported that the plasma α-KG concentration was increased in the patients with chronic hepatic dysfunctions, Reye’s syndrome and fulminent hepatic failure1,2. On the other hand, Batshaw et al.3 reported that plasma α-KG level was decreased in a hyperammonemic state in the patients with various urea cycle enzymopathies. In the patients with urea cycle enzymopathies, the citric acid cycle (TCA cycle) which is closely linked to the urea cycle, might be significantly influenced due to excessive ammonia. Thus it seems important to study the changes of the TCA cycle components in the urea cycle enzymopathy.

Keywords

Glutamate Dehydrogenase Urea Cycle Plasma Ammonium Excessive Ammonia Ornithine TRANSCARBAMYLASE 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

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References

  1. 1.
    A.M. Dawson, J. Groote, W.S. Rosenthal and S. Sherlock, Blood pyruvic-acid and a-ketoglutaric acid levels in liver disease and hepatic coma, Lancet 1: 392 (1957).CrossRefGoogle Scholar
  2. 2.
    C.O. Record, R.A. Iles, R.D. Cohen, and R. Williams, Acid-base and metabolic disturbances in fulminant hepatic failure, Gut 16: 144 (1975).PubMedCrossRefGoogle Scholar
  3. 3.
    M.L. Batshaw, M. Walser, and S.W. Brusilow, Plasma a-ketoglutarate in urea cycle enzymopathies and its role as a harbinger of hyperammonemic coma, Pediatr. Res. 14: 1316 (1980).Google Scholar
  4. 4.
    L.E. Rogers and S.F. Porter, Hereditary orotic aciduria. II A urinary screening test, Pediatrics 42: 423 (1968).PubMedGoogle Scholar
  5. 5.
    C.T. Nuzum, and P.J. Snodgrass, Multiple assays of the five urea-cycle enzymes in human liver homogenates, in: “The urea cycle”, S. Grisolia, R. Baguena, and F. Mayor, ed., Wiley & Sons, New York, (1976).Google Scholar
  6. 6.
    M. Nakajima, and Y. Ozawa, A highly efficient carboxylic acid analyser and its application, J. Chromatogr. 123: 129 (1976).PubMedCrossRefGoogle Scholar
  7. 7.
    H.U. Bergmeyer, and E. Bernt, 2-oxoglutarate, in: “Methods of enzymatic analysis”, H.U. Bergmeyer ed., Academic Press, New York (1974).Google Scholar
  8. 8.
    R.W. Bonsnes, and H.H. Tausshey, On the colorimetric determination of creatinine by the Jaffe reaction, J. Biol. Chem. 158: 581 (1945).Google Scholar
  9. 9.
    J.T. Brosnan, and D.H. Williamson, Mechanisms for the formation of alanine and aspartate on rat liver in vivo after administration of ammonium chloride, Biochem. J. 138: 453 (1974).PubMedGoogle Scholar
  10. 10.
    B. Hindfelt, The effect of substained hyperammonemia upon the metabolic state of the brain, Scand. J. Clin. Lab. Invest. 30: 245 (1972).PubMedCrossRefGoogle Scholar
  11. 11.
    M.L. Batshaw and S.W. Brusilow, Asymptomatic hyperammonemia in low birthweight infants, Pediatr. Res. 12: 221 (1978).Google Scholar
  12. 12.
    S.P. Bessman and A.N. Bessman, The cerebral and peripheral uptake of ammonia in liver disease with an hypothesis for the mechanism of hepatic coma, J. Clin. Invest. 34: 622 (1955).PubMedCrossRefGoogle Scholar
  13. 13.
    B. Levin, J.M. Abraham, V.G. Oberholzer and E.A. Burgess, Hyperammonemia. A deficiency of liver ornithine trans-carbamylase, Arch. Dis. Childh. 44: 152 (1969).CrossRefGoogle Scholar
  14. 14.
    P. Sunshine, J.E. Lendenbaum, H.L. Levy and J.M. Freeman, Hyperammonemia due to a defect in hepatic ornithine transcarbamylase, Pediatrics 50: 100 (1972).PubMedGoogle Scholar
  15. 15.
    C. Heiden, H.D. Bakker, J. Desplanque, M. Brink, P.K. de Bee, and S.K. Wadman, Attempted dietary treatment of a boy with hyperammonemia due to ornithine transcarbamylase deficiency, Eur. J. Pediatr. 128: 261 (1978).PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • Hiroko Kodama
    • 1
  • Osamu Nose
    • 1
  • Shintaro Okada
    • 1
  • Hyakuji Yabuuchi
    • 1
  1. 1.Department of PediatricsOsaka University School of MedicineFukushima-ku, OsakaJapan

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