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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References in periodicals archive ?
Reed, "A trail of research from lipoic acid to alpha-keto acid dehydrogenase complexes," The Journal of Biological Chemistry, vol.
Alpha-keto acid dehydrogenase complexes: organization, regulation, and biomedical ramifications.
(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. (17,18) R-lipoic acid delivered in the plasma can cross the blood brain barrier and be reduced to DHLA.