An acid containing a ketone group (-CO-) in addition to the acid group(s); alpha-keto acid refers to a 2-oxo acid (e.g., pyruvic acid); beta-keto acid refers to a 3-oxo acid (e.g., acetoacetic acid), etc.
Synonym: oxo acid.
Alpha-keto acid dehydrogenase, one of several distinct multienzyme complexes that catalyses the formation of an acyl-CoA derivative, CO2, and NADH from an alpha-keto acid, NAD+, and coenzyme A; maple syrup urine disease results from several different inherited defects in the mitochondrial branched chain alpha-keto acid dehydrogenase complex.
(05 Mar 2000)