Isovaleryl-CoA: Difference between revisions
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Revision as of 05:00, 3 March 2025
Isovaleryl-CoA is an intermediate in the metabolism of leucine, an essential amino acid in humans. It is a substrate for the enzyme isovaleryl-CoA dehydrogenase, which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA.
Metabolism
Leucine is an essential amino acid that is metabolized in the body to produce energy. The first step in the metabolism of leucine is the transamination of leucine to α-ketoisocaproate by the enzyme branched-chain amino acid transaminase. α-Ketoisocaproate is then decarboxylated to isovaleryl-CoA by the enzyme branched-chain alpha-keto acid dehydrogenase complex.
Isovaleryl-CoA is then converted to 3-methylcrotonyl-CoA by the enzyme isovaleryl-CoA dehydrogenase. This reaction is a part of the leucine degradation pathway, which ultimately leads to the production of acetyl-CoA, a key molecule in energy production.
Clinical significance
Mutations in the gene encoding isovaleryl-CoA dehydrogenase can lead to isovaleric acidemia, a rare metabolic disorder characterized by a deficiency of isovaleryl-CoA dehydrogenase. This can result in an accumulation of isovaleryl-CoA and its metabolites in the body, leading to various symptoms such as poor feeding, vomiting, lethargy, and in severe cases, coma or death.
