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. 1993 Feb;68(2):202–204. doi: 10.1136/adc.68.2.202

Abnormal expression of glucose-6-phosphatase in preterm infants.

R Hume 1, A Burchell 1
PMCID: PMC1029235  PMID: 8386919

Abstract

The hepatic microsomal glucose-6-phosphatase enzyme was studied in liver samples from 76 premature infants including 15 victims of sudden infant death syndrome. The data obtained were compared with glucose-6-phosphatase activity in liver samples from 95 term infants. In the majority of preterm infants up to 350 days of age the activity of the glucose-6-phosphatase enzyme was at or below the extreme low limit of the normal range in term infants. The premature infants with the lowest hepatic microsomal glucose-6-phosphatase activities are likely to be at risk of hypoglycaemic episodes during periods of relative starvation or stress.

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Selected References

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

  1. Allison F., Bennett M. J., Variend S., Engel P. C. Acylcoenzyme A dehydrogenase deficiency in heart tissue from infants who died unexpectedly with fatty change in the liver. Br Med J (Clin Res Ed) 1988 Jan 2;296(6614):11–12. doi: 10.1136/bmj.296.6614.11. [DOI] [PMC free article] [PubMed] [Google Scholar]
  2. Blair J. N., Burchell A. The mechanism of histone activation of the hepatic microsomal glucose-6-phosphatase system: a novel method to assay glucose-6-phosphatase activity. Biochim Biophys Acta. 1988 Feb 17;964(2):161–167. doi: 10.1016/0304-4165(88)90162-6. [DOI] [PubMed] [Google Scholar]
  3. Burchell A., Bell J. E., Busuttil A., Hume R. Hepatic microsomal glucose-6-phosphatase system and sudden infant death syndrome. Lancet. 1989 Aug 5;2(8658):291–294. doi: 10.1016/s0140-6736(89)90484-4. [DOI] [PubMed] [Google Scholar]
  4. Burchell A., Burchell B. Stabilization of partially-purified glucose 6-phosphatase by fluoride. Is enzyme inactivation caused by dephosphorylation? FEBS Lett. 1980 Sep 8;118(2):180–184. doi: 10.1016/0014-5793(80)80214-6. [DOI] [PubMed] [Google Scholar]
  5. Burchell A., Gibb L., Waddell I. D., Giles M., Hume R. The ontogeny of human hepatic microsomal glucose-6-phosphatase proteins. Clin Chem. 1990 Sep;36(9):1633–1637. [PubMed] [Google Scholar]
  6. Burchell A., Hume R., Burchell B. A new microtechnique for the analysis of the human hepatic microsomal glucose-6-phosphatase system. Clin Chim Acta. 1988 Apr 15;173(2):183–191. doi: 10.1016/0009-8981(88)90256-2. [DOI] [PubMed] [Google Scholar]
  7. Burchell A., Jung R. T., Lang C. C., Bennet W., Shepherd A. N. Diagnosis of type 1a and type 1c glycogen storage diseases in adults. Lancet. 1987 May 9;1(8541):1059–1062. doi: 10.1016/s0140-6736(87)90484-3. [DOI] [PubMed] [Google Scholar]
  8. Burchell A., Leakey J. E. Development of the rat hepatic microsomal glucose-6-phosphatase system and its glucocorticoid inducibility. Biol Neonate. 1988;54(2):107–115. doi: 10.1159/000242832. [DOI] [PubMed] [Google Scholar]
  9. Coughtrie M. W., Blair J. N., Hume R., Burchell A. Improved preparation of hepatic microsomes for in vitro diagnosis of inherited disorders of the glucose-6-phosphatase system. Clin Chem. 1991 May;37(5):739–742. [PubMed] [Google Scholar]
  10. Emery J. L., Howat A. J., Variend S., Vawter G. F. Investigation of inborn errors of metabolism in unexpected infant deaths. Lancet. 1988 Jul 2;2(8601):29–31. doi: 10.1016/s0140-6736(88)92955-8. [DOI] [PubMed] [Google Scholar]
  11. Hallock J., Morrow G., 3rd, Karp L. A., Barness L. A. Postmortem diagnosis of metabolic disorders. The finding of maple syrup urine disease in a case of sudden and unexpected death in infancy. Am J Dis Child. 1969 Oct;118(4):649–651. [PubMed] [Google Scholar]
  12. Harpey J. P., Charpentier C., Coudé M., Divry P., Paturneau-Jouas M. Sudden infant death syndrome and multiple acyl-coenzyme A dehydrogenase deficiency, ethylmalonic-adipic aciduria, or systemic carnitine deficiency. J Pediatr. 1987 Jun;110(6):881–884. doi: 10.1016/s0022-3476(87)80401-8. [DOI] [PubMed] [Google Scholar]
  13. Harpey J. P., Charpentier C., Paturneau-Jouas M. Fatty acid beta-oxidation defects and sudden infant death. Lancet. 1987 Jan 17;1(8525):163–163. doi: 10.1016/s0140-6736(87)92000-9. [DOI] [PubMed] [Google Scholar]
  14. Howat A. J., Bennett M. J., Variend S., Shaw L. Deficiency of medium chain fatty acylcoenzyme A dehydrogenase presenting as the sudden infant death syndrome. Br Med J (Clin Res Ed) 1984 Mar 31;288(6422):976–976. doi: 10.1136/bmj.288.6422.976. [DOI] [PMC free article] [PubMed] [Google Scholar]
  15. Howat A. J., Bennett M. J., Variend S., Shaw L., Engel P. C. Defects of metabolism of fatty acids in the sudden infant death syndrome. Br Med J (Clin Res Ed) 1985 Jun 15;290(6484):1771–1773. doi: 10.1136/bmj.290.6484.1771. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Pears J. S., Jung R. T., Hopwood D., Waddell I. D., Burchell A. Glycogen storage disease diagnosed in adults. Q J Med. 1992 Mar;82(299):207–222. [PubMed] [Google Scholar]
  17. Peterson G. L. A simplification of the protein assay method of Lowry et al. which is more generally applicable. Anal Biochem. 1977 Dec;83(2):346–356. doi: 10.1016/0003-2697(77)90043-4. [DOI] [PubMed] [Google Scholar]
  18. Polak J. M., Wigglesworth J. S. Letter: Islet-cell hyperplasia and sudden infant death. Lancet. 1976 Sep 11;2(7985):570–571. doi: 10.1016/s0140-6736(76)91814-6. [DOI] [PubMed] [Google Scholar]
  19. Russell M. A., Opitz J. M., Viseskul C., Gilbert E. F., Bargman G. J. Sudden infant death due to congenital adrenal hypoplasia. Arch Pathol Lab Med. 1977 Apr;101(4):168–169. [PubMed] [Google Scholar]
  20. Sturner W. Q., Susa J. B. Sudden infant death and liver phosphoenolpyruvate carboxykinase analysis. Forensic Sci Int. 1980 Jul-Aug;16(1):19–28. doi: 10.1016/0379-0738(80)90175-9. [DOI] [PubMed] [Google Scholar]

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