| methylcrotonoyl-CoA carboxylase |
[EC 6.4.1.4] a biotin-containing enzyme of the ligase class that catalyzes the ATP-driven carboxylation of 3-methylcrotonyl CoA to form 3-methylglutaconyl CoA as a step in the use of leucine as a fuel. Deficiency of the enzyme, an autosomal recessive trait, results in 3-methylcrotonyl carboxylase deficiency; enzyme activity is also absent in multiple carboxylase deficiency (q.v.). Written also methylcrotonyl CoA carboxylase.
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