Peroxisomal acyl-coenzyme A oxidase 3 (ACOX3)

Peroxisomal acyl-coenzyme A oxidase 3 (ACOX3) is an enzyme localized in peroxisomes, catalyzing the first and rate-limiting step in the β-oxidation of branched-chain and 2-methyl-branched fatty acids. It oxidizes CoA-esters of these substrates by transferring electrons via FAD to molecular oxygen, producing hydrogen peroxide that is subsequently detoxified by catalase. ACOX3 is crucial for lipid metabolism and redox balance. Unlike the rat analog, human ACOX3 is expressed at low levels in liver, possibly being active primarily in other tissues or under special conditions. Its deficiency or dysfunction is implicated in metabolic disorders such as adrenoleukodystrophy and Zellweger syndrome.

Other names

Acyl-CoA oxidase 3Pristanoyl-CoA oxidaseBranched-chain acyl-CoA oxidaseBRCOXPRCOXBRCACoxAcyl-Coenzyme A oxidase 3, pristanoyl

Catalysis of the α,β-dehydrogenation of acyl-CoA substrates in peroxisomal β-oxidation