ACOX1

Acyl-CoA oxidase 1, palmitoyl
Identifiers
Symbols	ACOX1 ; ACOX; PALMCOX; SCOX
External IDs	OMIM: 609751 MGI: 1330812 HomoloGene: 38299 GeneCards: ACOX1 Gene
EC number	1.3.3.6
Gene ontology Molecular function • fatty-acyl-CoA binding • acyl-CoA dehydrogenase activity • acyl-CoA oxidase activity • receptor binding • electron carrier activity • palmitoyl-CoA oxidase activity • PDZ domain binding • protein N-terminus binding • flavin adenine dinucleotide binding • oxidoreductase activity, acting on the CH-CH group of donors, with a flavin as acceptor • FAD binding Cellular component • nucleus • nucleoplasm • nucleolus • mitochondrion • peroxisome • peroxisomal membrane • peroxisomal matrix • plasma membrane • membrane • intracellular membrane-bounded organelle Biological process • very long-chain fatty acid metabolic process • generation of precursor metabolites and energy • lipid metabolic process • prostaglandin metabolic process • spermatogenesis • peroxisome fission • fatty acid oxidation • fatty acid beta-oxidation using acyl-CoA dehydrogenase • fatty acid beta-oxidation using acyl-CoA oxidase • unsaturated fatty acid metabolic process • alpha-linolenic acid metabolic process • cellular lipid metabolic process • small molecule metabolic process • lipid homeostasis • positive regulation of cholesterol homeostasis Sources: Amigo / QuickGO
RNA expression pattern
More reference expression data
Orthologs
Species	Human	Mouse
Entrez	51	11430
Ensembl	ENSG00000161533	ENSMUSG00000020777
UniProt	Q15067	Q9R0H0
RefSeq (mRNA)	NM_001185039	NM_001271898
RefSeq (protein)	NP_001171968	NP_001258827
Location (UCSC)	Chr 17: 75.94 – 75.98 Mb	Chr 11: 116.17 – 116.2 Mb
PubMed search

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

References

1. Varanasi U, Chu R, Chu S, Espinosa R, LeBeau MM, Reddy JK (May 1994). "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. doi:10.1073/pnas.91.8.3107. PMC 43524. PMID 8159712.
2. "Entrez Gene: ACOX1 acyl-Coenzyme A oxidase 1, palmitoyl".

Further reading

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