dihydrolipoyl dehydrogenase

di·hy·dro·lip·o·yl de·hy·dro·gen·ase (di-hi″dro-lipґo-əl de-hiґdro-jən-ās) [EC 1.8.1.4] an enzyme of the oxidoreductase class that catalyzes the regeneration of lipoyl groups from dihydrolipoyl groups, reducing NAD⁺. The enzyme contains FAD and is a component of several oxidative decarboxylation enzyme complexes, including the branched-chain α-keto acid dehydrogenase, α-ketoglutarate dehydrogenase, and pyruvate dehydrogenase complexes; deficiency of the enzyme, an autosomal recessive trait, is a cause of maple syrup urine disease.