Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027960 (mole)
 21,279 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Using a fraction purified from liver peroxisomes, we demonstrate that products of the glutaryl-CoA oxidase reaction are glutaconyl-CoA and H2O2. No glutaconyl-CoA decarboxylation occurs with this fraction. In whole tissue homogenates, the handling of glutaryl-CoA by glutaryl-CoA dehydrogenase is inhibited when reoxidation of FADH2 is blocked. Under these conditions, glutaconyl-CoA decarboxylation, however, can still occur and 14CO2 is produced from labelled glutaryl-CoA in mole/mole ratio with H2O2. These data indicate that in the absence of its mitochondrial dehydrogenation, glutaryl-CoA is oxidized in peroxisomes to glutaconyl-CoA which is probably transferred to mitochondria where it is decarboxylated and further processed. This hypothesis allows coherent explanation for the observed organic aciduria in both glutaricaciduria types I and II.
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PMID:Mitochondrial and peroxisomal metabolism of glutaryl-CoA. 397 69