Very long-chain acyl-CoA synthetase

Very long-chain acyl-CoA synthetase (VLACS) is an enzyme that plays a crucial role in the metabolism of fatty acids. It is responsible for the activation of very long-chain fatty acids, a process that is essential for their subsequent metabolism.

Function

VLACS catalyzes the initial step in the metabolism of fatty acids, which is the conversion of a fatty acid to an acyl-CoA molecule. This process involves the addition of a two-carbon unit, the CoA, to the fatty acid. The resulting acyl-CoA molecule can then be further metabolized, either through beta-oxidation to produce energy, or incorporated into complex lipids such as phospholipids and triglycerides.

Structure

VLACS is a member of the acyl-CoA synthetase family of enzymes. These enzymes share a common structure, with a large catalytic domain that contains the active site, and a smaller AMP-binding domain that binds the AMP molecule required for the reaction.

Clinical significance

Mutations in the gene encoding VLACS can lead to a rare metabolic disorder known as very long-chain acyl-CoA dehydrogenase deficiency (VLCADD). This condition is characterized by an inability to metabolize very long-chain fatty acids, leading to a buildup of these substances in the body. Symptoms can include hypoglycemia, cardiomyopathy, and muscle weakness.

See also

• Acyl-CoA synthetase
• Fatty acid metabolism
• Very long-chain acyl-CoA dehydrogenase deficiency