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Medical Term:

a-keto acid dehydrogenase


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

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