3-Methylglutaconyl-CoA

3-Methylglutaconyl-CoA is a key intermediate in the metabolism of leucine, an essential amino acid. It is produced by the enzyme 3-methylglutaconyl-CoA hydratase, and its accumulation in the body can lead to a rare metabolic disorder known as 3-Methylglutaconic aciduria.

Biochemical Role

In the metabolic pathway of leucine, 3-Methylglutaconyl-CoA is converted into 3-Hydroxy-3-methylglutaryl-CoA (HMG-CoA) by the enzyme 3-methylglutaconyl-CoA hydratase. This reaction is part of the leucine degradation pathway, which is essential for the breakdown and utilization of leucine in the body.

Clinical Significance

A deficiency in the enzyme 3-methylglutaconyl-CoA hydratase can lead to an accumulation of 3-Methylglutaconyl-CoA and its metabolites in the body. This condition is known as 3-Methylglutaconic aciduria, a rare inborn error of metabolism. Symptoms can include developmental delay, muscle weakness, and cardiomyopathy.

See Also

• Leucine
• 3-Methylglutaconic aciduria
• 3-Hydroxy-3-methylglutaryl-CoA
• 3-methylglutaconyl-CoA hydratase