Skip to main content
SpringerLink
Log in

The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria

  • JSIMD Meeting
  • Published:
Journal of Inherited Metabolic Disease

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

References

  • Gatfield, P. D., Taller, E., Wolfe, D. M. and Haust, M. D. Hyperornithinaemia, hyperammonaemia and homocitrullinuria associated with decreased carbamyl phosphate synthetase I activity.Pediatr. Res. 9 (1975) 488–497

    Article  PubMed  CAS  Google Scholar 

  • Hommes, F. A., Ho, C. K., Roesel, R. A. and Coryell, M. E. Decreased transport of ornithine across the inner mitochondrial membrane as a cause of hyperornithinaemia.J. Inher. Metab. Dis. 5 (1982) 41–47

    Article  PubMed  CAS  Google Scholar 

  • Shih, V. E., Efron, M. L. and Moser, H. W. Hyperornithinemia, hyperammonemia and homocitrullinuria. A new disorder of amino acid metabolism associated with myoclonic seizures and mental retardation.Am. J. Dis. Child. 177 (1969) 83–92

    Google Scholar 

  • Shih, V. E., Mandell, R. and Herzfeld, A. Defective ornithine metabolism in cultured skin fibroblasts from patients with the syndrome of hyperornithinemia, hyperammonemia and homocitrullinuria.Clin. Chim. Acta 118 (1982) 149–157

    Article  PubMed  CAS  Google Scholar 

  • Wright, T. and Pollitt, R. Psychomotor retardation, epileptic and stuporous attacks, irritability and ataxia associated with ammonia intoxication, high blood ornithine levels and increased homocitrulline in the urine.Proc. R. Soc. Med. 66 (1973) 221–226

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Sapporo Medical College, 060, Sapporo, Japan

    K. Oyanagi, A. Tsuchiyama, Y. Itakura, H. Sogawa, K. Wagatsuma & T. Nakao

  2. Department of Internal Medicine, Sapporo Medical College, 060, Sapporo, Japan

    S. Sakamoto & A. Yachi

Authors
  1. K. Oyanagi
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. Tsuchiyama
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Y. Itakura
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. H. Sogawa
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. K. Wagatsuma
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. T. Nakao
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. S. Sakamoto
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. A. Yachi
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Oyanagi, K., Tsuchiyama, A., Itakura, Y. et al. The mechanism of hyperammonaemia and hyperornithinaemia in the syndrome of hyperornithinaemia, hyperammonaemia with homocitrullinuria. J Inherit Metab Dis 6, 133–134 (1983). https://doi.org/10.1007/BF01800748

Download citation

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF01800748

Keywords

Search

Navigation