Hepatic polyamine metabolism in children with Reye's syndrome.
Acute mitochondrial insult has been suggested as a primary reason for the clinical, histopathological and biochemical abnormalities seen in Reye's syndrome. However, the etiology of mitochondrial dysfunction has not been identified. Polyamines have been known to alter the mitochondrial structure and function. Influenza infection may cause an increase in ornithine decarboxylase activity and thereby channel ornithine for polyamine biosynthesis, leading to mitochondrial dysfunction in Reye's syndrome. To test this hypothesis, the hepatic concentrations of polyamines, polyamine-metabolizing enzymes and urea cycle enzyme activities in Reye's syndrome patients were determined and compared with patients who died from illnesses other than Reye's syndrome. The hepatic concentration of putrescine, spermidine and spermine were increased in Reye's syndrome patients. The activity of ornithine decarboxylase was elevated but, due to the small number of samples, these values did not reach statistical significance. Ornithine carbamoyltransferase activity was decreased in the liver of Reye's syndrome patients. Our results suggest that increased synthesis of polyamines from ornithine may initiate mitochondrial injury in Reye's syndrome.
Mukhopadhyay
A
A
Department of Pediatrics, Children's Hospital of Michigan, Wayne State University, Detroit.
Deshmukh
D R
DR
Sarnaik
A P
AP
D002648:Child; D006801:Humans; D007668:Kidney / Q000201:enzymology; D008099:Liver / Q000378:metabolism*; D009954:Ornithine Carbamoyltransferase / Q000032:analysis; D009955:Ornithine Decarboxylase / Q000032:analysis; D011073:Polyamines / Q000378:metabolism*; D011700:Putrescine / Q000032:analysis; D012202:Reye Syndrome / Q000201:enzymology / Q000378:metabolism*; D013095:Spermidine / Q000032:analysis; D013096:Spermine / Q000032:analysis; D014508:Urea / Q000378:metabolism
D011073:Polyamines; D013096:Spermine; D014508:Urea; D009954:Ornithine Carbamoyltransferase; D009955:Ornithine Decarboxylase; D013095:Spermidine; D011700:Putrescine