Gene Symbol: Acox1
Description: acyl-Coenzyme A oxidase 1, palmitoyl
Alias: AOX, Acox, D130055E20Rik, Paox, peroxisomal acyl-coenzyme A oxidase 1, palmitoyl-CoA oxidase, peroxisomal fatty acyl-coenzyme A oxidase, peroxisomal straight-chain Acyl-CoA oxidase
Species: mouse
Products:     Acox1

Top Publications

  1. Fan C, Pan J, Chu R, Lee D, Kluckman K, Usuda N, et al. Hepatocellular and hepatic peroxisomal alterations in mice with a disrupted peroxisomal fatty acyl-coenzyme A oxidase gene. J Biol Chem. 1996;271:24698-710 pubmed
    ..The biologic role of peroxisomal beta-oxidation system, which consists of three enzymes: fatty acyl-CoA oxidase (ACOX), enoyl-CoA hydratase/3-hydroxyacyl-CoA dehydrogenase (HD), and thiolase, has been examined in mice by disrupting ..
  2. Fan C, Pan J, Usuda N, Yeldandi A, Rao M, Reddy J. Steatohepatitis, spontaneous peroxisome proliferation and liver tumors in mice lacking peroxisomal fatty acyl-CoA oxidase. Implications for peroxisome proliferator-activated receptor alpha natural ligand metabolism. J Biol Chem. 1998;273:15639-45 pubmed
    ..The first step of this system, catalyzed by acyl-CoA oxidase (AOX), converts fatty acyl-CoA to 2-trans-enoyl-CoA...
  3. Huang J, Viswakarma N, Yu S, Jia Y, Bai L, Vluggens A, et al. Progressive endoplasmic reticulum stress contributes to hepatocarcinogenesis in fatty acyl-CoA oxidase 1-deficient mice. Am J Pathol. 2011;179:703-13 pubmed publisher
    Fatty acyl-coenzyme A oxidase 1 (ACOX1) knockout (ACOX1(-/-)) mice manifest hepatic metabolic derangements that lead to the development of steatohepatitis, hepatocellular regeneration, spontaneous peroxisome proliferation, and ..
  4. Hashimoto T, Fujita T, Usuda N, Cook W, Qi C, Peters J, et al. Peroxisomal and mitochondrial fatty acid beta-oxidation in mice nullizygous for both peroxisome proliferator-activated receptor alpha and peroxisomal fatty acyl-CoA oxidase. Genotype correlation with fatty liver phenotype. J Biol Chem. 1999;274:19228-36 pubmed to respond to the inductive effects of peroxisome proliferators, whereas those lacking fatty acyl-CoA oxidase (AOX-/-), the first enzyme of the peroxisomal beta-oxidation system, exhibit extensive microvesicular steatohepatitis, ..
  5. Yeldandi A, Rao M, Reddy J. Hydrogen peroxide generation in peroxisome proliferator-induced oncogenesis. Mutat Res. 2000;448:159-77 pubmed
    ..Mice with disrupted fatty acyl-CoA oxidase gene (AOX(-/-) mice), which encodes the first enzyme of the PPARalpha regulated peroxisomal beta-oxidation system, exhibit ..
  6. Meyer K, Lee J, Dyck P, Cao W, Rao M, Thorgeirsson S, et al. Molecular profiling of hepatocellular carcinomas developing spontaneously in acyl-CoA oxidase deficient mice: comparison with liver tumors induced in wild-type mice by a peroxisome proliferator and a genotoxic carcinogen. Carcinogenesis. 2003;24:975-84 pubmed
    ..profiles of 26 hepatocellular carcinomas (HCC) developing spontaneously in peroxisomal fatty acyl-CoA oxidase null (AOX-/-) mice...
  7. Huang J, Jia Y, Fu T, Viswakarma N, Bai L, Rao M, et al. Sustained activation of PPAR? by endogenous ligands increases hepatic fatty acid oxidation and prevents obesity in ob/ob mice. FASEB J. 2012;26:628-38 pubmed publisher
    ..Mice lacking acyl-CoA oxidase 1 (Acox1), the first enzyme of the peroxisomal fatty acid ?-oxidation system, are characterized by increased energy ..
  8. Brites P, Motley A, Gressens P, Mooyer P, Ploegaert I, Everts V, et al. Impaired neuronal migration and endochondral ossification in Pex7 knockout mice: a model for rhizomelic chondrodysplasia punctata. Hum Mol Genet. 2003;12:2255-67 pubmed
    ..These findings demonstrate that Pex7 knockout mice provide an important model to study the role of peroxisomal functioning in the pathogenesis of the human disorder...
  9. Meyer K, Jia Y, Cao W, Kashireddy P, Rao M. Expression of peroxisome proliferator-activated receptor alpha, and PPARalpha regulated genes in spontaneously developed hepatocellular carcinomas in fatty acyl-CoA oxidase null mice. Int J Oncol. 2002;21:1175-80 pubmed
    Fatty acyl-CoA oxidase null mice (AOX-/-) develop hepatocellular carcinomas in 100% of animals between 10 and 15 months...

More Information


  1. Albrecht P, Torsell N, Krishnan P, Ehresman D, Frame S, Chang S, et al. A species difference in the peroxisome proliferator-activated receptor ?-dependent response to the developmental effects of perfluorooctanoic acid. Toxicol Sci. 2013;131:568-82 pubmed publisher
    ..Expression of mRNA encoding the PPAR? target genes acyl CoA oxidase (Acox1) and cytochrome P450 4a10 (Cyp4a10) in maternal and fetal liver was increased on GD18 in wild-type and PPAR?-..
  2. Infante J, Tschanz C, Shaw N, Michaud A, Lawrence P, Brenna J. Straight-chain acyl-CoA oxidase knockout mouse accumulates extremely long chain fatty acids from alpha-linolenic acid: evidence for runaway carousel-type enzyme kinetics in peroxisomal beta-oxidation diseases. Mol Genet Metab. 2002;75:108-19 pubmed
    ..Straight-chain acyl-CoA oxidase (AOX) catalyzes the first, rate-limiting step of peroxisomal beta-oxidation of very-long-chain saturated and unsaturated ..
  3. Kim S, Marquard K, Stephens S, Louden E, Allsworth J, Moley K. Adiponectin and adiponectin receptors in the mouse preimplantation embryo and uterus. Hum Reprod. 2011;26:82-95 pubmed publisher
    ..PCR, mRNA transcripts for Adipoq, AdipoR1, AdipoR2, Ppara, Ppard, FATP1 (SLC27A1) and acyl CoA oxidase (Acox1) were identified in mouse 2-cell and 8-cell embryos, while blastocyst stage embryos and trophoblast stem (TS) ..
  4. Hashimoto T, Cook W, Qi C, Yeldandi A, Reddy J, Rao M. Defect in peroxisome proliferator-activated receptor alpha-inducible fatty acid oxidation determines the severity of hepatic steatosis in response to fasting. J Biol Chem. 2000;275:28918-28 pubmed
    ..of hepatic steatosis in mice deficient in PPARalpha (PPARalpha(-/-)), peroxisomal fatty acyl-CoA oxidase (AOX(-/-)), and in both PPARalpha and AOX (double knock-out (DKO))...
  5. Riordan J, Nadeau J. Modeling progressive non-alcoholic fatty liver disease in the laboratory mouse. Mamm Genome. 2014;25:473-86 pubmed publisher
    ..Additionally, an overview of nutritional models of progressive NAFLD is provided. ..
  6. Lehman J, Barger P, Kovacs A, Saffitz J, Medeiros D, Kelly D. Peroxisome proliferator-activated receptor gamma coactivator-1 promotes cardiac mitochondrial biogenesis. J Clin Invest. 2000;106:847-56 pubmed
    ..These results identify PGC-1 as a critical regulatory molecule in the control of cardiac mitochondrial number and function in response to energy demands. ..
  7. Nohammer C, El Shabrawi Y, Schauer S, Hiden M, Berger J, Forss Petter S, et al. cDNA cloning and analysis of tissue-specific expression of mouse peroxisomal straight-chain acyl-CoA oxidase. Eur J Biochem. 2000;267:1254-60 pubmed
    ..Our data show that acyl-CoA oxidase is an evolutionary highly conserved enzyme with a distinct pattern of expression and indicate an important role in lipid metabolism. ..
  8. Baarine M, Andreoletti P, Athias A, Nury T, Zarrouk A, Ragot K, et al. Evidence of oxidative stress in very long chain fatty acid--treated oligodendrocytes and potentialization of ROS production using RNA interference-directed knockdown of ABCD1 and ACOX1 peroxisomal proteins. Neuroscience. 2012;213:1-18 pubmed publisher
    ..ATP-binding cassette transporter D (ABCD1) and from single peroxisomal enzyme deficiency (Acyl-CoA oxidase1: ACOX1), respectively...
  9. Gao Q, Jia Y, Yang G, Zhang X, Boddu P, Petersen B, et al. PPARα-Deficient ob/ob Obese Mice Become More Obese and Manifest Severe Hepatic Steatosis Due to Decreased Fatty Acid Oxidation. Am J Pathol. 2015;185:1396-408 pubmed publisher
    ..However, it does not lead to liver tumor development because of reduction in FAO and energy burning. ..
  10. Sheridan R, Lampe K, Shanmukhappa S, Putnam P, Keddache M, Divanovic S, et al. Lampe1: an ENU-germline mutation causing spontaneous hepatosteatosis identified through targeted exon-enrichment and next-generation sequencing. PLoS ONE. 2011;6:e21979 pubmed publisher
    ..whereas one mutation affected a splice donor site in intron 11-12 of Palmitoyl Acetyl-coenzyme A oxygenase-1 (Acox1), causing skipping of exon 12...
  11. Agbaga M, Mandal M, Anderson R. Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein. J Lipid Res. 2010;51:1624-42 pubmed publisher
    ..We focus on a novel elongase, ELOVL4, involved in the synthesis of VLC-PUFA, and the importance of these FAs in maintaining the structural and functional integrity of retinal photoreceptors. ..
  12. Li J, Viswanadha S, Loor J. Hepatic Metabolic, Inflammatory, and Stress-Related Gene Expression in Growing Mice Consuming a Low Dose of Trans-10, cis-12-Conjugated Linoleic Acid. J Lipids. 2012;2012:571281 pubmed publisher
    ..of genes associated with VLDL assembly/secretion (Mttp, Cideb), ketogenesis (Hmgcs2, Bdh1), and LCFA oxidation (Acox1, Pdk4) in response to trans-10, cis-12-CLA...
  13. Lee J, Chu I, Mikaelyan A, Calvisi D, Heo J, Reddy J, et al. Application of comparative functional genomics to identify best-fit mouse models to study human cancer. Nat Genet. 2004;36:1306-11 pubmed
    ..Gene expression patterns in HCCs from Acox1(-/-) mice and in ciprofibrate-induced HCCs were least similar to those observed in human HCCs...
  14. Huyghe S, Casteels M, Janssen A, Meulders L, Mannaerts G, Declercq P, et al. Prenatal and postnatal development of peroxisomal lipid-metabolizing pathways in the mouse. Biochem J. 2001;353:673-80 pubmed
  15. Berger J, Truppe C, Neumann H, Forss Petter S. cDNA cloning and mRNA distribution of a mouse very long-chain acyl-CoA synthetase. FEBS Lett. 1998;425:305-9 pubmed
  16. Guo D, Sarkar J, Suino Powell K, Xu Y, Matsumoto K, Jia Y, et al. Induction of nuclear translocation of constitutive androstane receptor by peroxisome proliferator-activated receptor alpha synthetic ligands in mouse liver. J Biol Chem. 2007;282:36766-76 pubmed
    ..These observations, therefore, provide information for the first time to indicate that PPARalpha ligands not only serve as PPARalpha agonists but possibly act as CAR antagonists. ..
  17. Nenicu A, Lüers G, Kovacs W, David M, Zimmer A, Bergmann M, et al. Peroxisomes in human and mouse testis: differential expression of peroxisomal proteins in germ cells and distinct somatic cell types of the testis. Biol Reprod. 2007;77:1060-72 pubmed
    ..Our results suggest a cell type-specific metabolic function of peroxisomes in the testis and point to an important role for peroxisomes in spermiogenesis and in the lipid metabolism of Sertoli cells. ..
  18. Takeshita S, Moritani M, Kunika K, Inoue H, Itakura M. Diabetic modifier QTLs identified in F2 intercrosses between Akita and A/J mice. Mamm Genome. 2006;17:927-40 pubmed
    ..Identification of the genes responsible for these QTLs would provide new drug development targets for human T2D. ..
  19. Cook W, Jain S, Jia Y, Cao W, Yeldandi A, Reddy J, et al. Peroxisome proliferator-activated receptor alpha-responsive genes induced in the newborn but not prenatal liver of peroxisomal fatty acyl-CoA oxidase null mice. Exp Cell Res. 2001;268:70-6 pubmed
    Mice deficient in fatty acyl-CoA oxidase (AOX(-/-)), the first enzyme of the peroxisomal beta-oxidation system, develop specific morphological and molecular changes in the liver characterized by microvesicular fatty change, increased ..
  20. Grabenbauer M, Fahimi H, Baumgart E. Detection of peroxisomal proteins and their mRNAs in serial sections of fetal and newborn mouse organs. J Histochem Cytochem. 2001;49:155-64 pubmed
    ..the developmental changes in localization of the peroxisomal marker enzymes catalase (CAT) and acyl-CoA oxidase 1 (AOX), the key regulatory enzyme of peroxisomal beta-oxidation, at the protein and mRNA levels in mice from embryonic ..
  21. Bass N. Three for the price of one knockout--a mouse model of a congenital peroxisomal disorder, steatohepatitis, and hepatocarcinogenesis. Hepatology. 1999;29:606-8 pubmed
  22. Osmundsen H, Landin M, From S, Kolltveit K, Risnes S. Changes in gene-expression during development of the murine molar tooth germ. Arch Oral Biol. 2007;52:803-13 pubmed
    ..Such data is therefore useful in directing attention towards genes, or cellular activities, which likely are worthy of further studies as regards their involvement in odontogenesis...
  23. Coulter A, Stephens J. STAT5 activators modulate acyl CoA oxidase (AOX) expression in adipocytes and STAT5A binds to the AOX promoter in vitro. Biochem Biophys Res Commun. 2006;344:1342-5 pubmed
    ..We also identified seven putative STAT elements in the murine AOX promoter. We observed that GH modulates protein binding to the majority of these promoter elements...
  24. Abbott B, Wood C, Watkins A, Tatum Gibbs K, Das K, Lau C. Effects of perfluorooctanoic acid (PFOA) on expression of peroxisome proliferator-activated receptors (PPAR) and nuclear receptor-regulated genes in fetal and postnatal CD-1 mouse tissues. Reprod Toxicol. 2012;33:491-505 pubmed publisher
    ..The metabolic disruption produced by PFOA may contribute to poor postnatal survival and persistent weight deficits of CD-1 mouse neonates. ..
  25. Vluggens A, Andreoletti P, Viswakarma N, Jia Y, Matsumoto K, Kulik W, et al. Reversal of mouse Acyl-CoA oxidase 1 (ACOX1) null phenotype by human ACOX1b isoform [corrected]. Lab Invest. 2010;90:696-708 pubmed publisher
    Disruption of the peroxisomal acyl-CoA oxidase 1 (Acox1) gene in the mouse results in the development of severe microvesicular hepatic steatosis and sustained activation of peroxisome proliferator-activated receptor-alpha (PPARalpha)...
  26. Tzeng J, Byun J, Park J, Yamamoto T, Schesing K, Tian B, et al. An Ideal PPAR Response Element Bound to and Activated by PPARα. PLoS ONE. 2015;10:e0134996 pubmed publisher
    ..Taken altogether, the provided PPRE sequence definition contributes to the understanding of PPARα signaling by identifying PPARα direct target genes with functional PPARα response elements. ..
  27. de Jong H, Neal A, Coleman R, Lewin T. Ontogeny of mRNA expression and activity of long-chain acyl-CoA synthetase (ACSL) isoforms in Mus musculus heart. Biochim Biophys Acta. 2007;1771:75-82 pubmed
    ..Our data support the hypothesis that each ACSL has a specific role in the channeling of FA towards distinct metabolic fates. ..
  28. Yubero P, Hondares E, Carmona M, Rossell M, Gonzalez F, Iglesias R, et al. The developmental regulation of peroxisome proliferator-activated receptor-gamma coactivator-1alpha expression in the liver is partially dissociated from the control of gluconeogenesis and lipid catabolism. Endocrinology. 2004;145:4268-77 pubmed
  29. Yu S, Rao S, Reddy J. Peroxisome proliferator-activated receptors, fatty acid oxidation, steatohepatitis and hepatocarcinogenesis. Curr Mol Med. 2003;3:561-72 pubmed
    ..Evidence from mice lacking fatty acyl-CoA oxidase (AOX), PPAR alpha and PPAR alpha/AOX has confirmed the role of PPAR alpha in the development of hepatocellular ..
  30. Berger J, Albet S, Bentejac M, Netik A, Holzinger A, Roscher A, et al. The four murine peroxisomal ABC-transporter genes differ in constitutive, inducible and developmental expression. Eur J Biochem. 1999;265:719-27 pubmed
    ..The mouse ALDR gene uses a single transcription start site but alternative polyadenylation sites. These data are of importance for the use of ALDP-deficient mice as a model in pharmacological gene therapy studies...
  31. Janssen A, Gressens P, Grabenbauer M, Baumgart E, Schad A, Vanhorebeek I, et al. Neuronal migration depends on intact peroxisomal function in brain and in extraneuronal tissues. J Neurosci. 2003;23:9732-41 pubmed
  32. Banks A, Kim Muller J, Mastracci T, Kofler N, Qiang L, Haeusler R, et al. Dissociation of the glucose and lipid regulatory functions of FoxO1 by targeted knockin of acetylation-defective alleles in mice. Cell Metab. 2011;14:587-97 pubmed publisher
    ..Thus, acetylation inactivates FoxO1 during nutrient excess whereas deacetylation selectively potentiates FoxO1 activity, protecting against excessive catabolism during nutrient deprivation. ..