Multiple enzymes; canonical full names for individual key enzymes: Acyl-CoA dehydrogenase, Enoyl-CoA hydratase, Hydroxyacyl-CoA dehydrogenase, Ketoacyl-CoA thiolase (None for this group; individually, abbreviations include: ACAD, EH, 3HAD, KAT)

Fatty acid β-oxidation pathway enzymes are a class of enzymes responsible for the stepwise breakdown of fatty acids in the mitochondria and peroxisomes. This process converts long-chain fatty acids into acetyl-CoA, NADH, and FADH2, facilitating entry into the tricarboxylic acid (citric acid) cycle and oxidative phosphorylation for ATP synthesis. The main enzymes involved are acyl-CoA dehydrogenase, enoyl-CoA hydratase, hydroxyacyl-CoA dehydrogenase, and ketoacyl-CoA thiolase[1][5]. Each has several isoforms specific for different chain lengths (e.g., short-, medium-, and long-chain acyl-CoA dehydrogenases)[1][3]. Dysfunction of any of these components can result in severe metabolic disturbances, including hypoglycemia, muscle weakness, coma, and death[2][4]. The pathway is also critical for the metabolism of very-long-chain and branched fatty acids via peroxisomal variants of these enzymes[2][4][1]. While the pathway is a key therapeutic target in rare diseases, most interventions focus on dietary and metabolic management rather than direct enzyme modulation. If a specific enzyme in the pathway (such as medium-chain acyl-CoA dehydrogenase, MCAD) is intended as the target, it should be explicitly named for structured data extraction.

Other names

Beta-oxidation enzymesFatty acid oxidation enzymesFatty acid β-oxidation complexIndividual aliases (e.g., MCAD for medium-chain acyl-CoA dehydrogenase, VLCAD for very-long-chain acyl-CoA dehydrogenase)

Activation: Drugs may upregulate enzymes to increase fatty acid oxidation (e.g., clofibrate). Inhibition: Theoretical: Inhibitors would decrease fatty acid breakdown, risking toxicity and energy deficit. Targeting transport/activation: Carnitine modulators (for enzymes like CPT1/2, not the core β-oxidation enzymes)