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Medical Term:

3-methylcrotonyl-CoA carboxylase


Definition: 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.

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