methylcrotonoyl-CoA carboxylase
- methylcrotonoyl-CoA carboxylase
- meth·yl·cro·ton·o·yl-CoA car·box·y·lase (meth″əl-kro-tonґo-əl ko-aґ kahr-bokґsə-lās) [EC 6.4.1.4] a biotin-containing enzyme of the ligase class that catalyzes the ATP-driven carboxylation of 1. 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.
Medical dictionary.
2011.
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