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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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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.

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