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Deficient activity of hepatic pyruvate dehydrogenase and pyruvate carboxylase in Reye's syndrome.

作者信息

Robinson B H, Gall D G, Cutz E

出版信息

Pediatr Res. 1977 Apr;11(4):279-81. doi: 10.1203/00006450-197704000-00003.

DOI:10.1203/00006450-197704000-00003
PMID:191789
Abstract

The activity of certain hepatic enzymes involved in carbohydrate metabolism was measured in postmortem samples from six cases of Reye's syndrome. The activities of the two exclusively extramitochondrial enzymes, glucose-6-phosphatase and fructose-1,6-diphosphatase, were all within the normal range. Activities of pyruvate carboxylase and pyruvate dehydrogenase, both of which are exclusively mitochondrial enzymes, were below levels, shown by control tissue in every case, the average being 21.7% of the lowest control value for pyruvate carboxylase and 11.6% of that for pyruvate dehydrogenase. Impaired pyruvate metabolism appears to be another feature in Reye's syndrome.

摘要

相似文献

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引用本文的文献

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Interactions of aspirin and other potential etiologic factors in an animal model of Reye syndrome.阿司匹林与瑞氏综合征动物模型中其他潜在病因因素的相互作用。
Proc Natl Acad Sci U S A. 1982 Dec;79(23):7557-60. doi: 10.1073/pnas.79.23.7557.
2
Neonatal pyruvate carboxylase deficiency with renal tubular acidosis and cystinuria.新生儿丙酮酸羧化酶缺乏伴肾小管酸中毒和胱氨酸尿症。
J Inherit Metab Dis. 1983;6(3):89-94. doi: 10.1007/BF01800731.
3
Disorders of the pyruvate dehydrogenase complex.丙酮酸脱氢酶复合体紊乱
J Inherit Metab Dis. 1986;9(2):105-19. doi: 10.1007/BF01799447.
4
Biochemical relationships between Reye's and Reye's-like metabolic and toxicological syndromes.瑞氏综合征及类瑞氏代谢与毒理学综合征之间的生化关系。
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Recurrent, familial Reye-like syndrome with a new complex amino and organic aciduria.伴有新型复合氨基酸和有机酸尿症的复发性家族性雷氏样综合征。
Eur J Pediatr. 1990 Jul;149(10):709-12. doi: 10.1007/BF01959528.