3-methylcrotonyl-CoA

3-methylcrotonyl-CoA
An intermediate in the degradation of l-leucine; accumulates in a deficiency of M. carboxylase.
- 3-methylcrotonyl-CoA carboxylase a biotin-dependent enzyme in the pathway of l-leucine degradation that catalyzes the reaction of M. with CO2, ATP, and water to form ADP, orthophosphate, and 3-methylglutaconyl-CoA; a deficiency of this enzyme causes episodes of severe metabolic acidosis.

Medical dictionary. 2011.

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