Peroxisomal beta-oxidation enzyme

Peroxisomal beta-oxidation enzymes are a group of **peroxisomal matrix enzymes responsible for the breakdown of very long chain fatty acids, branched-chain fatty acids, and bile acid intermediates** through beta-oxidation, a cyclical process that shortens fatty acyl-CoAs by two carbons per cycle[1][2][3][7]. The core group comprises: - **Acyl-CoA oxidase 1 (ACOX1):** catalyzes the first dehydrogenation step, producing hydrogen peroxide[4][7]. - **Bifunctional proteins (L- and D-):** carry out hydration and dehydrogenation of the β-oxidation spiral, acting on straight or branched substrates, respectively[7]. - **3-ketoacyl-CoA thiolase:** mediates thiolytic cleavage releasing acetyl-CoA[7]. - **Branched chain acyl-CoA oxidase, sterol carrier protein x:** act on specific fatty acid substrates such as pristanic acid and bile acid intermediates[7]. These enzymes are essential for lipid catabolism, energy homeostasis, and limiting accumulation of toxic fatty acid intermediates. Regulation is under control of PPARα, especially for classical pathway enzymes involved in straight-chain fatty acid degradation[2]. Pathological deficiency or dysregulation results in lipid storage disorders, metabolic syndromes, neurological deficits, or progressive liver disease[2][4][5]. Peroxisomal beta-oxidation is therapeutically manipulated by drugs like fibrates and targeted antioxidants[2][4]. **Note:** The query refers to a whole enzyme class/system rather than a specific protein, so "Peroxisomal beta-oxidation enzyme" is the most appropriate canonical singular form. For structured therapeutic databases, entries for individual enzymes (e.g., ACOX1) are preferred over the general class, so this target name is too broad for single-entity mapping and is considered *incorrect/over-broad* for most structured drug-target use cases.

Other names

Beta-oxidation enzyme (peroxisome)Peroxisomal fatty acid oxidation enzymeACOX1 (acyl-CoA oxidase 1)Bifunctional protein (L- and D-)Sterol carrier protein xPalmitoyl-CoA oxidaseBranched chain acyl-CoA oxidase3-ketoacyl-CoA thiolase

Drugs may act by **inducing enzyme expression** (e.g., hypolipidemic fibrates via PPARα agonism increases expression of classical pathway enzymes) Antioxidants may **attenuate ROS toxicity** generated by peroxisomal beta-oxidation