Peroxisomal acyl-coenzyme A oxidase 2 (ACOX2)

**Peroxisomal acyl-coenzyme A oxidase 2 (ACOX2)** is a peroxisomal flavoprotein enzyme that catalyzes the first and rate-limiting step in the β-oxidation of branched-chain fatty acids and of bile acid intermediates, particularly the CoA esters of di- and tri-hydroxycholestanoic acids[5][6][7]. It is crucial for bile acid biosynthesis and metabolic homeostasis, especially in the liver and kidney. ACOX2 deficiency results in impaired bile acid synthesis and accumulation of atypical C27 bile acids, leading to metabolic liver disease and is also implicated in tumor biology where it may act as a metabolic tumor suppressor[2][4]. There are currently no clinically approved drugs directly targeting ACOX2, but its activity and expression are being evaluated as biomarkers and potential therapeutic nodes for metabolic and hepatic diseases[2][4][5][6].

Other names

Branched chain acyl-CoA oxidaseBranched chain acyl-coenzyme A oxidaseBranched chain acyl-CoA oxidase 2THCA-CoA oxidaseTrihydroxycoprostanoyl-CoA oxidase3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA 24-hydroxylase3-alpha,7-alpha,12-alpha-trihydroxy-5-beta-cholestanoyl-CoA oxidasePeroxisomal branched chain acyl-CoA oxidaseCBAS6BCOXBRCACOXBRCOX

Putative small molecule activators or gene therapies (for deficiency) Potential modulation via pathways such as PPARα signaling[2][6]