Reactome | ACOX1 oxidizes C26:0 CoA

ACOX1 oxidizes C26:0 CoA

Stable Identifier

R-HSA-390256

Type

Reaction [transition]

Species

Homo sapiens

Compartment

peroxisomal matrix

Synonyms

hexacosanoyl-CoA + O2 => (2E)-hexacosenoyl-CoA + H2O2

ReviewStatus

5/5

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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
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, RJA, Waterham, HR	Hum Mutat	2007
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