A family of mitochondrial enzymes (acyl-coenzyme A dehydrogenases) that catalyse the first dehydrogenation step in the beta-oxidation of fatty acyl-CoA derivatives. There are different ACADs for short-, medium-, long- and very long-chain fatty acids. The product of the ACAD8 gene, also known as isobutyryl-coenzyme A (CoA) dehydrogenase (EC 1.3.99, 415 aa), converts isobutyryl-CoA into succinyl-CoA. At least four mutations in the ACAD8 gene have been identified and lead to isobutyryl-CoA dehydrogenase deficiency in which there may be dilated cardiomyopathy, anaemia, and carnitine deficiency.
Subjects: Medicine and Health.