Isovaleryl-CoA

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.

See also

• Leucine
• Isovaleric acidemia
• Branched-chain amino acid transaminase
• Branched-chain alpha-keto acid dehydrogenase complex
• Isovaleryl-CoA dehydrogenase

• 
  Isovaleryl coenzyme A