3-methylcrotonyl-CoA carboxylase


Also found in: Acronyms.

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.
References in periodicals archive ?
3-Methylglutaconyl-CoA hydratase, 3-methylcrotonyl-CoA carboxylase and 3-hydroxy-3-methylglutarylCoA lyase deficiencies: a coupled enzyme assay useful for their detection.
C50H is increased in 3-methylcrotonyl-CoA carboxylase deficiency, 3-hydroxy-3-methylglutaryl-CoA lyase deficiency, and 3-methylglutaconyl-CoA hydratase deficiency, whereas 2-methyl-3-hydroxybutyrylcarnitine is increased in [beta]-ketothiolase deficiency and 2-methyl-3-hydroxybutyrl-CoA dehydrogenase deficiency (118).
[beta]-KETOTHIOLASE, 3-HYDROXY-3-METHYLGLUTARYL-COA LYASE (HMG), AND 3-METHYLCROTONYL-COA CARBOXYLASE (MCC) DEFICIENCY AND MCD

Full browser ?