Methylglutaconyl-CoA

Methylglutaconyl-CoA is an intermediate in the metabolism of leucine, an essential amino acid. It is converted to hydroxymethylglutaryl-CoA (HMG-CoA) by the enzyme methylglutaconyl-CoA hydratase (AUH). This reaction is part of the leucine degradation pathway.

Structure

Methylglutaconyl-CoA is a coenzyme A derivative. It has a methyl group attached to the second carbon of the glutaconyl group, and a thioester bond linking it to coenzyme A.

Function

In the body, methylglutaconyl-CoA is involved in the breakdown of leucine. This process is important for energy production, as leucine is one of the three branched-chain amino acids (BCAAs), which are particularly significant in muscle protein synthesis and energy production.

Metabolism

Methylglutaconyl-CoA is converted to HMG-CoA by the enzyme AUH. This reaction is part of the leucine degradation pathway, which ultimately leads to the production of acetyl-CoA, a key molecule in energy metabolism.

Clinical significance

Mutations in the AUH gene, which codes for the enzyme that converts methylglutaconyl-CoA to HMG-CoA, can lead to 3-Methylglutaconic aciduria, a rare metabolic disorder characterized by increased levels of 3-methylglutaconic acid and 3-methylglutaric acid in the urine.

See also

• Leucine
• Coenzyme A
• Methylglutaconyl-CoA hydratase
• 3-Methylglutaconic aciduria

Methylglutaconyl-CoA Resources
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