alpha-keto acid dehydrogenase

(redirected from a-keto acid dehydrogenase)

α-ke·to ac·id de·hy·dro·gen·ase

one of several distinct multienzyme complexes that catalyzes the formation of an acyl-CoA derivative, CO2, and NADH from an α-keto acid, NAD+, and coenzyme A; maple syrup urine disease results from several different inherited defects in the mitochondrial branched chain α-keto acid dehydrogenase complex.