Ornithine Transcarbamylase (OTC) in White Blood Cells and Jejunal Mucosa

  • N. Nagata
  • I. Akaboshi
  • J. Yamamoto
  • F. Endo
  • I. Matsuda
  • T. Katsuki
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 153)

Abstract

The presence of OTC in white blood cells was demonstrated by Wolfe and Gatfield(1) and Snodgrass et al.(2), but was denied by Rabier et al (3). OTC deficiency was diagnosed using white blood cells by Wolfe and Gatfield (1) and Krieger et al (4). On the other hand, Snodgrass et al (2) reported that OTC deficiency in the liver can not be inferred from the measurements of the enzyme’s activity in peripheral white blood cells, because the latter parameter was normal in their patients. The presence of OTC in jejunal mucosa and reliability of this material for detecting OTC deficiency were demonstrated by Levin et al (5) and Cathelineau et al (6). However, the nature of OTC in intestinal mucosa was not clearly defined. We would like to describe some kinetic studies on OTC in white blood cells and jejunal mucosa.

Keywords

White Blood Cell Lymphoid Cell Urea Cycle Jejunal Mucosa Peripheral White Blood Cell 
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.
    D.M. Wolfe, and Gatfield, P.D., Leukocyte urea cycle enzymes in hyperammonemia. Pediatr. Res. 9: 531. (1975).PubMedCrossRefGoogle Scholar
  2. 2.
    P.J. Snodgrass, P.S. Wappner, and I.K. Brandt., Letter to the Editor: White cell ornithine transcarbamylase activity cannot detect the liver enzyme deficiency. Pediatr. Res. 12: 873 (1978).Google Scholar
  3. 3.
    P. Rabier, L. Cathelineau, and P. Kamoum., Letter to the Editor: Lack of mitochondrial enzymes of urea cycle in human white blood cells. Pediatr. Res. 13: 207 (1979).Google Scholar
  4. 4.
    F. Krieger, C. Bachmann, W. Gronemeyer, and J. Cejka., Propionic acidemia and hyperlysinemia in a case with ornithine transcarbamylase deficiency. J. Clin. Endcrinol. Metab. 43: 796 (1976).CrossRefGoogle Scholar
  5. 5.
    B. Levin, V.G. Oherholzer, and L. Sinclair., Biochemical investigations of hyperammonemia. Lancet 2: 120 (1969).Google Scholar
  6. 6.
    L. Cathelineau, J. Saudubray, and C. Polonsyski., Heterogenous mutations of the structural gene of human ornithine carbamyltransferase as observed in five personal cases. Enzyme 18: 103 (1974).PubMedGoogle Scholar
  7. 7.
    A. BoyUm., Separation of leukocytes from blood and bone marrow. Scan. J. Clin. Lab. Invest. Suppl. 21: 77 (1968).CrossRefGoogle Scholar
  8. 8.
    I. Matsuda, J. Yamamoto, N. Nagata, N. Ninomiya, I. Akaboshi, H. Ohtsuka, and I. Katsuki., Lysosomal enzyme activity in cultured lymphoid cell lines. Clin. Chim. Acta. 80: 483 (1977).PubMedCrossRefGoogle Scholar
  9. 9.
    F. Sinatra, T. Yoshida, M. Applebaum, W. Mason, J. Hoogenraad, and P. Sunshine, K., Abnormalities of carbamyl phosphate synthetase and ornithine transcarbamylase activity in liver of patients with Reye’s syndrome. Pediatr. Res. 9: 829 (1975).PubMedGoogle Scholar
  10. 10.
    G.W. Jr. Brown, and P.P. Cohen., Comparative biochemistry of urea cycle synthesis. I. Methods for the quantitative assay of urea cycle enzymes in liver. J. Biol. Chem. 239: 1770 (1959).Google Scholar
  11. 11.
    I. Matsuda, S. Arashima, H. Nambu, Y. Takekoshi, and M. Anakura., Hyperammonemia due to a mutant enzyme of ornithine transcarbamylase. Pediatrics. 48: 595 (1971).PubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 1982

Authors and Affiliations

  • N. Nagata
    • 1
  • I. Akaboshi
    • 1
  • J. Yamamoto
    • 1
  • F. Endo
    • 1
  • I. Matsuda
    • 1
  • T. Katsuki
    • 2
  1. 1.Department of PediatricsKumamoto University Medical School860 Japan
  2. 2.Department of MicrobiologyKumamoto University Medical School860 Japan

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