Glutaconyl-CoA (Glutaconyl-coenzyme A) is a key metabolic intermediate in the mitochondrial catabolic pathways of lysine, hydroxylysine and tryptophan. Glutaconyl-CoA competitively inhibits wild-type glutaryl-CoA dehydrogenase ( GCDH ) with a K i value of 1.1 µM. Glutaconyl-CoA is generated via the oxidative decarboxylation of glutaryl-CoA catalyzed by GCDH, and is subsequently further metabolized. Glutaconyl-CoA can be used in the research of type I glutaric aciduria.
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