hexacosanoyl-CoA + O2 => trans-2,3-dehydrohexacosanoyl-CoA + H2O2

Stable Identifier
R-HSA-390256
Type
Reaction [transition]
Species
Homo sapiens
Compartment
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Peroxisomal ACOX1 catalyzes the reaction of hexacosanoyl-CoA and O2 to form trans-2,3-dehydrohexacosanoyl-CoA and H2O2. The active form of the enzyme is a homodimer with FAD as a cofactor (Chu et al. 1995). Mutations in the ACOX1 gene are asociated with accumulation of very long chain fatty acids. Two isoforms of ACOX1, generated by alternative splicing are known; a mutation affecting specifically the second isoform blocks the oxidation of very long chain fatty acids (Ferdinandusse et al. 2007).

Literature References
PubMed ID Title Journal Year
7876265 Overexpression and characterization of the human peroxisomal acyl-CoA oxidase in insect cells

Chu, R, Varanasi, U, Chu, S, Lin, Y, Usuda, N, Rao, MS, Reddy, JK

J Biol Chem 1995
17458872 Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency

Ferdinandusse, S, Denis, S, Hogenhout, EM, Koster, J, van Roermund, CW, IJlst, L, Moser, AB, Wanders, RJ, Waterham, HR

Hum Mutat 2007
Participants
Participant Of
Catalyst Activity
Catalyst Activity
Title
acyl-CoA oxidase activity of ACOX1 dimer [peroxisomal matrix]
Physical Entity
Activity
Orthologous Events
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