Isovaleryl-coenzyme A, also known as isovaleryl-CoA, is an intermediate in the metabolism of branched-chain amino acids.

Isovaleryl coenzyme A is an intermediate in leucine catabolism. Iv-CoA is synthesized from β-methylbutyric acid by the action of enzyme acyl CoA synthetase. The conversion of isovaleryl-CoA to methylcrotonyl-CoA is catalyzed by the enzyme isovaleryl-CoA dehydrogenase in leucine catabolism pathway.

Isovaleryl CoA dehydrogenase is the name of an enzyme in your body that helps you digest a part of some proteins called leucine. If the isovaleryl CoA dehydrogenase your baby has does not work well, they will have trouble breaking down proteins containing leucine.

Isovaleric acidemia is a hereditary metabolic disorder, caused by a change (mutation) in the gene encoding the enzyme isovaleryl-CoA dehydrogenase, resulting in deficient or absent activity. This enzyme is responsible for helping break down leucine, an amino acid, and its deficiency leads to a buildup of chemicals in the blood that cause symptoms.