See Also: 3-methylcrotonyl-CoA(medicine)
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3-methylcrotonyl-CoA (medicine)
3-methylcrotonyl-CoA
(CH3)2C==CHCOSCoA;an intermediate in the degradation of l-leucine; accumulates in a deficiency of 3-methylcrotonyl-CoA carboxylase.
3-methylcrotonyl-CoA carboxylase, an enzyme in the pathway of l-leucine degradation that catalyses 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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