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.
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Evaluation of 3-methylcrotonyl-CoA carboxylase deficiency detected by tandem mass spectrometry newborn screening.
beta]-ketothiolase deficiency 3-Methylcrotonylglycine 3-Methylcrotonyl-CoA carboxylase deficiency; multiple carboxylase deficiency; HMG-CoA lyase deficiency 3-Methylglutaconate (37-42) 3-Methylglutaconyl-CoA hydratase deficiency (methylglutaconic aciduria type I); HMG-CoA lyase deficiency; 3-methylglutaconic aciduria (other than type I); respiratory chain defects (e.
beta]-KETOTHIOLASE, 3-HYDROXY-3-METHYLGLUTARYL-COA LYASE (HMG), AND 3-METHYLCROTONYL-COA CARBOXYLASE (MCC) DEFICIENCY AND MCD

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