3-methylcrotonyl-CoA carboxylase

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3-meth·yl·cro·ton·yl-CoA car·box·yl·ase

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