2,4 Dienoyl-CoA reductase

2,4-Dienoyl-CoA reductase (DECR) is an enzyme that plays a crucial role in the fatty acid oxidation pathway, specifically in the metabolism of unsaturated fatty acids. This enzyme is involved in the process of converting polyunsaturated fatty acyl-CoA esters to their corresponding saturated acyl-CoA forms. The activity of 2,4-Dienoyl-CoA reductase is essential for the degradation of unsaturated fatty acids, which is a key step in the generation of energy in the form of adenosine triphosphate (ATP) in cells.

Function

2,4-Dienoyl-CoA reductase catalyzes the reduction of 2,4-dienoyl-CoA to trans-3-enoyl-CoA in the presence of NADPH. This reaction is a part of the peroxisomal and mitochondrial fatty acid β-oxidation pathways, which are critical for the breakdown of long-chain and polyunsaturated fatty acids. The enzyme ensures that the diverse range of unsaturated fatty acids obtained from the diet or synthesized de novo can be efficiently processed to produce energy.

Structure

The enzyme is encoded by the DECR1 gene in humans. It is a protein that is localized to the mitochondria and peroxisomes, indicating its role in the fatty acid oxidation pathway in these organelles. The structure of 2,4-Dienoyl-CoA reductase includes a FAD-binding domain and a NADPH-binding domain, which are essential for its enzymatic activity.

Clinical Significance

Mutations in the DECR1 gene can lead to metabolic disorders characterized by an inability to properly metabolize unsaturated fatty acids. This can result in the accumulation of toxic intermediates, which can affect normal cellular functions and lead to clinical symptoms. Although rare, deficiencies in 2,4-Dienoyl-CoA reductase activity have been associated with neuromuscular disorders and hypoglycemia.

Research

Research on 2,4-Dienoyl-CoA reductase has focused on understanding its role in fatty acid metabolism and its implications in metabolic diseases. Studies have also explored the potential of targeting this enzyme for therapeutic interventions in metabolic disorders and conditions associated with impaired fatty acid oxidation.

See Also

• Fatty acid metabolism
• Oxidative phosphorylation
• Metabolic disorder

External Links

• DECR1 gene on NCBI
• 2,4-Dienoyl-CoA reductase on UniProt