3-Methylcrotonyl CoA

3-Methylcrotonyl CoA is a key metabolic intermediate in the leucine degradation pathway, converted to 3-Methylglutaconyl CoA by 3-Methylcrotonyl CoA Carboxylase. Normally, 3-Methylglutaconyl CoA is catalyzed by 3-Methylglutaconyl-CoA Hydratase into 3-Hydroxy-3-methylglutaryl-CoA (HMG CoA). However, mutations in 3-Methylglutaconyl-CoA Hydratase lead to the accumulation of 3-Methylglutaconyl CoA, causing metabolic disorders associated with 3MGA-uria. 3-Methylcrotonyl CoA is useful for research into metabolic diseases related to the leucine degradation pathway[1].