Reye's syndrome: preservation of mitochondrial enzymes in brain and muscle compared with liver.
The activities of five mitochondrial enzymes tested in liver from patients with Reye's syndrome were measured. Citrate synthase, glutamic dehydrogenase, succinic dehydrogenase, pyruvate carboxylase, and pyruvate dehydrogenase were all outside of the range shown by control samples and well below them in activity. The activity of two extramitochondrial enzymes, glucose-6-phosphatase, which is a microsomal enzyme, and fructose-1,6-diphosphatase, which is a soluble enzyme, were in the normal range in samples from Reye's syndrome patients. In both muscle and brain the activities of the mitochondrial enzyme, citrate synthase, glutamic dehydrogenase, and succinic dehydrogenase were all within the control range. Pyruvate dehydrogenase was found to be normal in muscle from these patients.
D001921:Brain / Q000201:enzymology* / Q000648:ultrastructure; D002648:Child; D002950:Citrate (si)-Synthase / Q000378:metabolism; D005260:Female; D006597:Fructose-Bisphosphatase / Q000378:metabolism; D005952:Glucose-6-Phosphatase / Q000378:metabolism; D005969:Glutamate Dehydrogenase / Q000378:metabolism; D006801:Humans; D008297:Male; D008928:Mitochondria / Q000201:enzymology; D008930:Mitochondria, Liver / Q000201:enzymology* / Q000648:ultrastructure; D008931:Mitochondria, Muscle / Q000201:enzymology* / Q000648:ultrastructure; D011766:Pyruvate Carboxylase / Q000378:metabolism; D011768:Pyruvate Dehydrogenase Complex / Q000378:metabolism; D012202:Reye Syndrome / Q000201:enzymology* / Q000473:pathology; D013385:Succinate Dehydrogenase / Q000378:metabolism
D011768:Pyruvate Dehydrogenase Complex; D013385:Succinate Dehydrogenase; D005969:Glutamate Dehydrogenase; D002950:Citrate (si)-Synthase; D006597:Fructose-Bisphosphatase; D005952:Glucose-6-Phosphatase; D011766:Pyruvate Carboxylase