alpha-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.
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17) R-lipoic acid or its reduced form, DHLA, is located in mitochondrial membranes where it serves as an important coenzyme in alpha-keto acid dehydrogenases.