ACOX1 explained

Peroxisomal acyl-coenzyme A oxidase 1 is an enzyme that in humans is encoded by the ACOX1 gene.^{[1] [2]}

The protein encoded by this gene is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids. Alternatively spliced transcript variants encoding different isoforms have been identified.^[2]

See also

• ACOX3
• Acyl-CoA oxidase

Further reading

• Seedorf U, Ellinghaus P, Roch Nofer J . Sterol carrier protein-2. . Biochim. Biophys. Acta . 1486 . 1 . 45–54 . 2000 . 10856712 . 10.1016/s1388-1981(00)00047-0.
• Singh H, Brogan M, Johnson D, Poulos A . Peroxisomal beta-oxidation of branched chain fatty acids in human skin fibroblasts. . J. Lipid Res. . 33 . 11 . 1597–605 . 1993 . 10.1016/S0022-2275(20)41382-3 . 1464743 . free .
• Watkins PA, McGuinness MC, Raymond GV . Distinction between peroxisomal bifunctional enzyme and acyl-CoA oxidase deficiencies. . Ann. Neurol. . 38 . 3 . 472–7 . 1995 . 7668838 . 10.1002/ana.410380322 . 8189860 . etal.
• Chu R, Varanasi U, Chu S . Overexpression and characterization of the human peroxisomal acyl-CoA oxidase in insect cells. . J. Biol. Chem. . 270 . 9 . 4908–15 . 1995 . 7876265 . 10.1074/jbc.270.9.4908 . etal. free .
• Fournier B, Saudubray JM, Benichou B . Large deletion of the peroxisomal acyl-CoA oxidase gene in pseudoneonatal adrenoleukodystrophy. . J. Clin. Invest. . 94 . 2 . 526–31 . 1994 . 8040306 . 10.1172/JCI117365 . 296126 . etal.
• Aoyama T, Tsushima K, Souri M . Molecular cloning and functional expression of a human peroxisomal acyl-coenzyme A oxidase. . Biochem. Biophys. Res. Commun. . 198 . 3 . 1113–8 . 1994 . 8117268 . 10.1006/bbrc.1994.1158 . etal.
• Maruyama K, Sugano S . Oligo-capping: a simple method to replace the cap structure of eukaryotic mRNAs with oligoribonucleotides. . Gene . 138 . 1–2 . 171–4 . 1994 . 8125298 . 10.1016/0378-1119(94)90802-8 .
• Pacot C, Latruffe N . Biochemical properties of liver peroxisomes from rat, guinea pig and human species and the influence of hormonal status on rat liver acyl-CoA oxidase mRNA content . Biochimie . 75 . 3–4 . 235–42 . 1993 . 8507686 . 10.1016/0300-9084(93)90082-4 .
• Fan CY, Pan J, Chu R . Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene . J. Biol. Chem. . 271 . 40 . 24698–710 . 1996 . 8798738 . 10.1074/jbc.271.40.24698 . etal. free .
• Suzuki Y, Yoshitomo-Nakagawa K, Maruyama K . Construction and characterization of a full length-enriched and a 5'-end-enriched cDNA library . Gene . 200 . 1–2 . 149–56 . 1997 . 9373149 . 10.1016/S0378-1119(97)00411-3 . etal.
• Fujiwara C, Imamura A, Hashiguchi N . Catalase-less peroxisomes. Implication in the milder forms of peroxisome biogenesis disorder . J. Biol. Chem. . 275 . 47 . 37271–7 . 2001 . 10960480 . 10.1074/jbc.M006347200 . etal. free .
• Hartley JL, Temple GF, Brasch MA . DNA cloning using in vitro site-specific recombination . Genome Res. . 10 . 11 . 1788–95 . 2001 . 11076863 . 10.1101/gr.143000 . 310948 .
• Wiemann S, Weil B, Wellenreuther R . Toward a catalog of human genes and proteins: sequencing and analysis of 500 novel complete protein coding human cDNAs . Genome Res. . 11 . 3 . 422–35 . 2001 . 11230166 . 10.1101/gr.GR1547R . 311072 . etal.
• Suzuki Y, Iai M, Kamei A . Peroxisomal acyl CoA oxidase deficiency . J. Pediatr. . 140 . 1 . 128–30 . 2002 . 11815777 . 10.1067/mpd.2002.120511 . 36469133 . etal.
• Strausberg RL, Feingold EA, Grouse LH . Generation and initial analysis of more than 15,000 full-length human and mouse cDNA sequences . Proc. Natl. Acad. Sci. U.S.A. . 99 . 26 . 16899–903 . 2003 . 12477932 . 10.1073/pnas.242603899 . 139241 . etal . 2002PNAS...9916899M. free .
• Ota T, Suzuki Y, Nishikawa T . Complete sequencing and characterization of 21,243 full-length human cDNAs . Nat. Genet. . 36 . 1 . 40–5 . 2004 . 14702039 . 10.1038/ng1285 . etal. free .
• Gerhard DS, Wagner L, Feingold EA . The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC) . Genome Res. . 14 . 10B . 2121–7 . 2004 . 15489334 . 10.1101/gr.2596504 . 528928 . etal.
• Wiemann S, Arlt D, Huber W . From ORFeome to biology: a functional genomics pipeline . Genome Res. . 14 . 10B . 2136–44 . 2004 . 15489336 . 10.1101/gr.2576704 . 528930 . etal.

Notes and References

1. Varanasi U, Chu R, Chu S, Espinosa R, LeBeau MM, Reddy JK . Isolation of the human peroxisomal acyl-CoA oxidase gene: organization, promoter analysis, and chromosomal localization . Proc Natl Acad Sci U S A . 91 . 8 . 3107–11 . May 1994 . 8159712 . 43524 . 10.1073/pnas.91.8.3107 . 1994PNAS...91.3107V . free .
2. Web site: Entrez Gene: ACOX1 acyl-Coenzyme A oxidase 1, palmitoyl.