FEATURES

FAMILY

DESCRIPTION

Also known as ACOX1_RAT, Acox1, Acox. Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. First enzyme of the fatty acid beta-oxidation pathway., Shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Shows optimum activity with a chain length of 10 carbons while isoform 2 exhibits optimum activity with 14 carbons., Is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1, 16-hexadecanodioyl-CoA. Homodimer. The enzyme contains three components A, B and C, the latter two being produced from the first by a proteolytic cleavage. Interacts with LONP2 (By similarity).

EXTERNAL LINKS

Acox1

Mutations

Mutation(s) distribution

Mutations table