异戊酰辅酶 A 是亮氨酸分解代谢的中间产物。Iv-CoA由β甲基丁酸在酯酰辅酶A合成酶的催化下而合成。在亮氨酸分解代谢途径中,异戊酰辅酶A脱氢酶将异戊酰辅酶A转化为甲基巴豆酰辅酶A。异戊酰辅酶A氧化酶同样也催化脂肪酸β-氧化途径中的这一转化。
Isovaleryl-coenzyme A (isovaleryl-CoA) is a branched-chain acyl CoA and an intermediate in the metabolism of the essential amino acid L-leucine. It is formed from L-leucine by the branched-chain α-keto acid dehydrogenase (BCKAD) complex and is converted to 3-methylcrotonyl-CoA by isovaleryl-CoA dehydrogenase (IVD), an enzyme involved in the inborn error of metabolism isovaleric acidemia. Isovaleryl-CoA increases the oxidation rate of the branched-chain 2-oxo acids 3-methyl-2-butanoate and 4-methyl-2-oxopentanoate in isolated rat muscle mitochondria in the absence of carnitine when used at a concentration of 1 mM. It also inhibits succinate-CoA ligase activity in rat liver mitochondria (IC50 = 273 µM).
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. The enzyme isovaleryl-CoA oxidase also calalyzes this conversion in fatty acid β-oxidation pathway.