Skip to main content
NCBI home page
Archives of Disease in Childhood logoLink to Archives of Disease in Childhood
. 1969 Dec;44(238):688–693. doi: 10.1136/adc.44.238.688

Carbamylphosphate synthetase deficiency in an infant with severe cerebral damage.

F A Hommes, C J De Groot, C W Wilmink, J H Jonxis
PMCID: PMC2020356  PMID: 5356974

Full text

PDF
688

Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. ALLAN J. D., CUSWORTH D. C., DENT C. E., WILSON V. K. A disease, probably hereditary characterised by severe mental deficiency and a constant gross abnormality of aminoacid metabolism. Lancet. 1958 Jan 25;1(7013):182–187. doi: 10.1016/s0140-6736(58)90666-4. [DOI] [PubMed] [Google Scholar]
  2. BROWN G. W., Jr, COHEN P. P. Comparative biochemistry of urea synthesis. I. Methods for the quantitative assay of urea cycle enzymes in liver. J Biol Chem. 1959 Jul;234(7):1769–1774. [PubMed] [Google Scholar]
  3. DANCIS J., HUTZLER J., LEVITZ M. Metabolism of the white blood cells in maple-syrup-urine disease. Biochim Biophys Acta. 1960 Sep 23;43:342–343. doi: 10.1016/0006-3002(60)90448-0. [DOI] [PubMed] [Google Scholar]
  4. ESTABROOK R. W., MAITRA P. K. A fluorimetric method for the quantitative microanalysis of adenine and pyridine nucleotides. Anal Biochem. 1962 May;3:369–382. doi: 10.1016/0003-2697(62)90065-9. [DOI] [PubMed] [Google Scholar]
  5. Floyd J. C., Jr, Fajans S. S., Conn J. W., Knopf R. F., Rull J. Stimulation of insulin secretion by amino acids. J Clin Invest. 1966 Sep;45(9):1487–1502. doi: 10.1172/JCI105456. [DOI] [PMC free article] [PubMed] [Google Scholar]
  6. Kemp J. W., Woodbury D. M. Synthesis of urea-cycle intermediates from citrulline in brain. Biochim Biophys Acta. 1965 Nov 15;111(1):23–31. doi: 10.1016/0304-4165(65)90469-1. [DOI] [PubMed] [Google Scholar]
  7. Levin B., Abraham J. M., Oberholzer V. G., Burgess E. A. Hyperammonaemia: a deficiency of liver ornithine transcarbamylase. Occurrence in mother and child. Arch Dis Child. 1969 Apr;44(234):152–161. doi: 10.1136/adc.44.234.152. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Levin B., Dobbs R. H., Burgess E. A., Palmer T. Hyperammonaemia. A variant type of deficiency of liver ornithine transcarbamylase. Arch Dis Child. 1969 Apr;44(234):162–169. doi: 10.1136/adc.44.234.162. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Levin B., Russell A. Treatment of hyperammonemia. Am J Dis Child. 1967 Jan;113(1):142–145. doi: 10.1001/archpedi.1967.02090160192031. [DOI] [PubMed] [Google Scholar]
  10. MOORE S., STEIN W. H. A modified ninhydrin reagent for the photometric determination of amino acids and related compounds. J Biol Chem. 1954 Dec;211(2):907–913. [PubMed] [Google Scholar]
  11. RUSSELL A., LEVIN B., OBERHOLZER V. G., SINCLAIR L. Hyperammonaemia. A new instance of an inborn enzymatic defect of the biosynthesis of urea. Lancet. 1962 Oct 6;2(7258):699–700. doi: 10.1016/s0140-6736(62)90508-1. [DOI] [PubMed] [Google Scholar]
  12. Räihä N. C., Suihkonen J. Development of urea-synthesizing enzymes in human liver. Acta Paediatr Scand. 1968 Mar;57(2):121–124. doi: 10.1111/j.1651-2227.1968.tb04663.x. [DOI] [PubMed] [Google Scholar]
  13. SHARMA C., MANJESHWAR R., WEINHOUSE S. EFFECTS OF DIET AND INSULIN ON GLUCOSE-ADENOSINE TRIPHOSPHATE PHOSPHOTRANSFERASES OF RAT LIVER. J Biol Chem. 1963 Dec;238:3840–3845. [PubMed] [Google Scholar]
  14. SPORN M. B., DINGMAN W., DEFALCO A., DAVIES R. K. Formation of urea from arginine in the brain of the living rat. Nature. 1959 May 30;183(4674):1520–1521. doi: 10.1038/1831520a0. [DOI] [PubMed] [Google Scholar]
  15. STEIN W. H., MOORE S. The free amino acids of human blood plasma. J Biol Chem. 1954 Dec;211(2):915–926. [PubMed] [Google Scholar]
  16. Walker D. G., Khan H. H., Eaton S. W. Enzymes catalysing the phosphorylation of hexoses in neonatal animals. Biol Neonat. 1965;9(1):224–239. [PubMed] [Google Scholar]

Articles from Archives of Disease in Childhood are provided here courtesy of BMJ Publishing Group

RESOURCES