- 3-methylcrotonyl-CoA
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- 3-methylcrotonyl-CoA carboxylase a biotin-dependent enzyme in the pathway of l-leucine degradation that catalyzes the reaction of M. with CO2, ATP, and water to form ADP, orthophosphate, and 3-methylglutaconyl-CoA; a deficiency of this enzyme causes episodes of severe metabolic acidosis.
Medical dictionary. 2011.