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Aliases for ACOX1 Gene

Aliases for ACOX1 Gene

  • Acyl-CoA Oxidase 1, Palmitoyl 2 3 5
  • Acyl-Coenzyme A Oxidase 1, Palmitoyl 2 3
  • Straight-Chain Acyl-CoA Oxidase 3 4
  • Palmitoyl-CoA Oxidase 3 4
  • EC 4 63
  • ACOX 3 4
  • SCOX 3 4
  • AOX 3 4
  • Peroxisomal Fatty Acyl-CoA Oxidase 3
  • Acyl-CoA Oxidase, Straight-Chain 3

External Ids for ACOX1 Gene

Previous GeneCards Identifiers for ACOX1 Gene

  • GC17P073433
  • GC17M076968
  • GC17M074401
  • GC17M074538
  • GC17M071453
  • GC17M073937
  • GC17M069363

Summaries for ACOX1 Gene

Entrez Gene Summary for ACOX1 Gene

  • 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. [provided by RefSeq, Jul 2008]

GeneCards Summary for ACOX1 Gene

ACOX1 (Acyl-CoA Oxidase 1, Palmitoyl) is a Protein Coding gene. Diseases associated with ACOX1 include Peroxisomal Acyl-Coa Oxidase Deficiency and Adrenoleukodystrophy. Among its related pathways are alpha-linolenic (omega3) and linoleic (omega6) acid metabolism and cAMP signaling pathway. GO annotations related to this gene include receptor binding and protein N-terminus binding. An important paralog of this gene is ACOX3.

UniProtKB/Swiss-Prot for ACOX1 Gene

  • Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.

Gene Wiki entry for ACOX1 Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for ACOX1 Gene

Genomics for ACOX1 Gene

Regulatory Elements for ACOX1 Gene

Enhancers for ACOX1 Gene
GeneHancer Identifier Score Enhancer Sources TSS distance (kb) Number of Genes Away Size (kb) Transcription Factor Binding Sites within enhancer Other Gene Targets for Enhancer

Enhancers around ACOX1 on UCSC Golden Path with GeneCards custom track

Promoters for ACOX1 Gene
Ensembl Regulatory Elements (ENSRs) TSS Distance (bp) Size (bp) Binding Sites for Transcription Factors within promoters

ENSRs around ACOX1 on UCSC Golden Path with GeneCards custom track

Genomic Location for ACOX1 Gene

75,941,507 bp from pter
75,979,434 bp from pter
37,928 bases
Minus strand

Genomic View for ACOX1 Gene

Genes around ACOX1 on UCSC Golden Path with GeneCards custom track

Cytogenetic band:
ACOX1 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
Genomic Location for ACOX1 Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for ACOX1 Gene

Proteins for ACOX1 Gene

  • Protein details for ACOX1 Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Recommended name:
    Peroxisomal acyl-coenzyme A oxidase 1
    Protein Accession:
    Secondary Accessions:
    • A8K6X8
    • A8KAA0
    • B4DK61
    • F5GYQ8
    • Q12863
    • Q15068
    • Q15101
    • Q16131
    • Q7Z3W5
    • Q9UD31

    Protein attributes for ACOX1 Gene

    660 amino acids
    Molecular mass:
    74424 Da
    Name=FAD; Xref=ChEBI:CHEBI:57692;
    Quaternary structure:
    No Data Available
    • Isoform 1 and isoform 2 can reverse the Acox1 null phenotype in mouse which is characterized by severe microvesicular hepatic steatosis, sustained activation of Ppara, spontaneous massive peroxisome proliferation and eventual development of hepatocellular carcinomas. Isoform 2 is more effective in reversal of the phenotype than isoform 1 (PubMed:20195242).
    • Sequence=CAD97622.1; Type=Erroneous initiation; Note=Translation N-terminally shortened.; Evidence={ECO:0000305};

    Alternative splice isoforms for ACOX1 Gene


neXtProt entry for ACOX1 Gene

Proteomics data for ACOX1 Gene at MOPED

Post-translational modifications for ACOX1 Gene

  • Modification sites at PhosphoSitePlus
  • Modification sites at neXtProt

Antibody Products

  • Abcam antibodies for ACOX1

Domains & Families for ACOX1 Gene

Graphical View of Domain Structure for InterPro Entry



  • Belongs to the acyl-CoA oxidase family.
  • Belongs to the acyl-CoA oxidase family.
genes like me logo Genes that share domains with ACOX1: view

No data available for Gene Families for ACOX1 Gene

Function for ACOX1 Gene

Molecular function for ACOX1 Gene

GENATLAS Biochemistry:
acyl-CoA oxidase,peroxisomal matrix,medium or long chain,catalyzing the first step of peroxisomal fatty acid beta-oxidation
UniProtKB/Swiss-Prot BiophysicochemicalProperties:
Kinetic parameters: KM=73 uM for palmitoyl-CoA (isoform 1) {ECO:0000269 PubMed:17603022}; KM=90 uM for palmitoyl-CoA (isoform 2) {ECO:0000269 PubMed:17603022}; pH dependence: Optimum pH is 8.5 for isoform 1 and 7.5-8.5 for isoform 2. {ECO:0000269 PubMed:17603022}; Temperature dependence: Optimum temperature for isoform 1 at pH 7.5 is 40 degrees Celsius with no activity at 50 degrees Celsius. Optimum temperature for isoform 2 at pH 7.5 is 47.5 degrees Celsius with 57% activity retained at 50 degrees Celsius. {ECO:0000269 PubMed:17603022};
UniProtKB/Swiss-Prot CatalyticActivity:
Acyl-CoA + O(2) = trans-2,3-dehydroacyl-CoA + H(2)O(2).
UniProtKB/Swiss-Prot Function:
Catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. Isoform 1 shows highest activity against medium-chain fatty acyl-CoAs and activity decreases with increasing chain length. Isoform 2 is active against a much broader range of substrates and shows activity towards very long-chain acyl-CoAs. Isoform 2 is twice as active as isoform 1 against 16-hydroxy-palmitoyl-CoA and is 25% more active against 1,16-hexadecanodioyl-CoA.

Enzyme Numbers (IUBMB) for ACOX1 Gene

Gene Ontology (GO) - Molecular Function for ACOX1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0000062 fatty-acyl-CoA binding IBA --
GO:0009055 electron carrier activity IBA --
GO:0016401 palmitoyl-CoA oxidase activity IDA 17603022
GO:0050660 flavin adenine dinucleotide binding IEA --
genes like me logo Genes that share ontologies with ACOX1: view
genes like me logo Genes that share phenotypes with ACOX1: view

Human Phenotype Ontology for ACOX1 Gene

HPO Id HPO Name Alternative Ids Definition Synonyms

Animal Models for ACOX1 Gene

MGI Knock Outs for ACOX1:

Animal Model Products

miRNA for ACOX1 Gene

miRTarBase miRNAs that target ACOX1

No data available for Transcription Factor Targets and HOMER Transcription for ACOX1 Gene

Localization for ACOX1 Gene

Subcellular locations from UniProtKB/Swiss-Prot for ACOX1 Gene


Subcellular locations from

Jensen Localization Image for ACOX1 Gene COMPARTMENTS Subcellular localization image for ACOX1 gene
Compartment Confidence
nucleus 5
peroxisome 5
plasma membrane 5
mitochondrion 3
cytosol 1

Gene Ontology (GO) - Cellular Components for ACOX1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IEA --
GO:0016020 membrane IDA 19946888
genes like me logo Genes that share ontologies with ACOX1: view

Pathways & Interactions for ACOX1 Gene

genes like me logo Genes that share pathways with ACOX1: view

UniProtKB/Swiss-Prot Q15067-ACOX1_HUMAN

  • Pathway: Lipid metabolism; peroxisomal fatty acid beta-oxidation.

Gene Ontology (GO) - Biological Process for ACOX1 Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006091 generation of precursor metabolites and energy IMP 7876265
GO:0006629 lipid metabolic process IDA 8117268
GO:0006693 prostaglandin metabolic process IMP 7876265
GO:0007283 spermatogenesis IEA --
GO:0008152 metabolic process IEA --
genes like me logo Genes that share ontologies with ACOX1: view

No data available for SIGNOR curated interactions for ACOX1 Gene

Drugs & Compounds for ACOX1 Gene

(22) Drugs for ACOX1 Gene - From: DrugBank, HMDB, and Novoseek

Name Status Disease Links Group Role Mechanism of Action Clinical Trials
FAD Approved Pharma Target 0
(3R)-3-HYDROXYDODECANOIC ACID Experimental Pharma Target 0
myristoyl-coa Experimental Pharma 0
Propionyl-CoA Experimental Pharma 0
Hydrogen peroxide Pharma 47

(65) Additional Compounds for ACOX1 Gene - From: Novoseek and HMDB

Name Synonyms Role CAS Number PubChem IDs PubMed IDs
  • Ac-CoA
  • Ac-Coenzyme A
  • Ac-S-CoA
  • Ac-S-Coenzyme A
  • Acetyl coenzyme-A
  • Dioxygen
  • Molecular oxygen
  • O2
  • Oxygen
  • Oxygen molecule
  • (E)-S-2-decenoate
  • (E)-S-2-decenoate CoA
  • (E)-S-2-decenoate Coenzyme A
  • (E)-S-2-decenoic acid
  • 2-trans-Decenoyl-CoA
  • (2E)-Dodec-2-enoyl-CoA
  • (2E)-Dodec-2-enoyl-Coenzyme A
  • 2-trans-Dodecenoyl-CoA
  • 2-trans-Dodecenoyl-Coenzyme A
  • (2E)-Hexadecenoyl-CoA
  • (2E)-Hexadecenoyl-Coenzyme A
  • trans-2-Hexadecenoyl-CoA
  • trans-2-Hexadecenoyl-Coenzyme A
genes like me logo Genes that share compounds with ACOX1: view

Transcripts for ACOX1 Gene

Unigene Clusters for ACOX1 Gene

Acyl-CoA oxidase 1, palmitoyl:
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for ACOX1 Gene

ExUns: 1a · 1b · 1c · 1d · 1e ^ 2 ^ 3 ^ 4 ^ 5a · 5b ^ 6a · 6b ^ 7 ^ 8 ^ 9 ^ 10 ^ 11 ^ 12 ^ 13 ^ 14 ^ 15a · 15b · 15c ^ 16
SP1: - -
SP2: - -
SP3: - - - -
SP4: - -
SP5: - -

Relevant External Links for ACOX1 Gene

GeneLoc Exon Structure for
ECgene alternative splicing isoforms for

Expression for ACOX1 Gene

mRNA expression in normal human tissues for ACOX1 Gene

Protein differential expression in normal tissues from HIPED for ACOX1 Gene

This gene is overexpressed in Cervix (12.1), Liver (7.7), Peripheral blood mononuclear cells (7.6), and Fetal Liver (6.3).

Integrated Proteomics: protein expression in normal tissues and cell lines from ProteomicsDB, PaxDb, MOPED, and MaxQB for ACOX1 Gene

SOURCE GeneReport for Unigene cluster for ACOX1 Gene Hs.464137

mRNA Expression by UniProt/SwissProt for ACOX1 Gene

Tissue specificity: Widely expressed with highest levels of isoform 1 and isoform 2 detected in testis. Isoform 1 is expressed at higher levels than isoform 2 in liver and kidney while isoform 2 levels are higher in brain, lung, muscle, white adipose tissue and testis. Levels are almost equal in heart.
genes like me logo Genes that share expression patterns with ACOX1: view

Protein tissue co-expression partners for ACOX1 Gene

Primer Products

No data available for mRNA expression in embryonic tissues and stem cells from LifeMap Discovery and mRNA differential expression in normal tissues for ACOX1 Gene

Orthologs for ACOX1 Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for ACOX1 Gene

Organism Taxonomy Gene Similarity Type Details
(Bos Taurus)
Mammalia ACOX1 35
  • 89.65 (n)
  • 91.97 (a)
ACOX1 36
  • 85 (a)
(Canis familiaris)
Mammalia ACOX1 35
  • 91.16 (n)
  • 93.33 (a)
ACOX1 36
  • 90 (a)
(Mus musculus)
Mammalia Acox1 35
  • 81.06 (n)
  • 84.39 (a)
Acox1 16
Acox1 36
  • 88 (a)
(Pan troglodytes)
Mammalia ACOX1 35
  • 99.55 (n)
  • 99.7 (a)
ACOX1 36
  • 100 (a)
(Rattus norvegicus)
Mammalia Acox1 35
  • 85.56 (n)
  • 88.64 (a)
(Monodelphis domestica)
Mammalia ACOX1 36
  • 92 (a)
(Ornithorhynchus anatinus)
Mammalia -- 36
  • 83 (a)
-- 36
  • 86 (a)
(Gallus gallus)
Aves ACOX1 35
  • 73.43 (n)
  • 77.27 (a)
ACOX1 36
  • 81 (a)
(Anolis carolinensis)
Reptilia ACOX1 36
  • 78 (a)
tropical clawed frog
(Silurana tropicalis)
Amphibia acox1 35
  • 66.26 (n)
  • 72.12 (a)
(Danio rerio)
Actinopterygii acox1 35
  • 67.02 (n)
  • 69.65 (a)
wufb59h12 35
acox1 36
  • 70 (a)
fruit fly
(Drosophila melanogaster)
Insecta Acox57D-d 37
  • 37 (a)
Acox57D-p 37
  • 38 (a)
BcDNA:GH07485 37
  • 43 (a)
CG4586 37
  • 38 (a)
CG5009 35
  • 52.54 (n)
  • 44.72 (a)
Acox57D-d 36
  • 36 (a)
Acox57D-p 36
  • 37 (a)
CG4586 36
  • 38 (a)
CG5009 36
  • 43 (a)
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP011798 35
  • 51.82 (n)
  • 43.77 (a)
(Caenorhabditis elegans)
Secernentea C48B4.1 37
  • 38 (a)
F08A8.1 37
  • 43 (a)
F08A8.2 37
  • 40 (a)
F08A8.3 37
  • 42 (a)
F08A8.4 37
  • 42 (a)
F25C8.1 37
  • 46 (a)
F59F4.1 37
  • 42 (a)
acox-1 35
  • 52.38 (n)
  • 45.03 (a)
acox-1 36
  • 42 (a)
C48B4.1 36
  • 38 (a)
F08A8.2 36
  • 42 (a)
F08A8.3 36
  • 42 (a)
F08A8.4 36
  • 42 (a)
F59F4.1 36
  • 42 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes POX1 36
  • 26 (a)
POX1 38
thale cress
(Arabidopsis thaliana)
eudicotyledons ACX1 35
  • 51.84 (n)
  • 45.11 (a)
(Glycine max)
eudicotyledons Gma.8460 35
(Oryza sativa)
Liliopsida Os.9192 35
Os06g0103500 35
  • 52.75 (n)
  • 44.24 (a)
(Triticum aestivum)
Liliopsida Ta.27205 35
(Zea mays)
Liliopsida Zm.4993 35
sea squirt
(Ciona intestinalis)
Ascidiacea Cin.4108 35
sea squirt
(Ciona savignyi)
Ascidiacea CSA.11092 36
  • 43 (a)
CSA.6819 36
  • 54 (a)
Species with no ortholog for ACOX1:
  • A. gosspyii yeast (Ashbya gossypii)
  • Actinobacteria (Mycobacterium tuberculosis)
  • African clawed frog (Xenopus laevis)
  • Alicante grape (Vitis vinifera)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • bread mold (Neurospora crassa)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • fission yeast (Schizosaccharomyces pombe)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • K. lactis yeast (Kluyveromyces lactis)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rainbow trout (Oncorhynchus mykiss)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)

Evolution for ACOX1 Gene

Gene Tree for ACOX1 (if available)
Gene Tree for ACOX1 (if available)

Paralogs for ACOX1 Gene

Paralogs for ACOX1 Gene

(3) SIMAP similar genes for ACOX1 Gene using alignment to 7 proteins:

genes like me logo Genes that share paralogs with ACOX1: view

Variants for ACOX1 Gene

Sequence variations from dbSNP and Humsavar for ACOX1 Gene

SNP ID Clin Chr 17 pos Sequence Context AA Info Type
rs1135640 - 75,953,459(-) GAAAT(C/G)AAGCC reference, missense
VAR_025789 Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD)
VAR_025790 Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD)
rs17855420 - 75,957,539(+) TGGCT(A/G)TGGTT reference, missense
rs3744032 - 75,973,754(+) GAGGC(C/T)CACAG intron-variant, reference, missense, utr-variant-5-prime

Structural Variations from Database of Genomic Variants (DGV) for ACOX1 Gene

Variant ID Type Subtype PubMed ID
dgv3264n71 CNV Loss 21882294
dgv3267n71 CNV Loss 21882294
nsv833545 CNV Gain 17160897
nsv908821 CNV Loss 21882294
esv2671149 CNV Deletion 23128226
nsv908824 CNV Loss 21882294
nsv908825 CNV Loss 21882294

Variation tolerance for ACOX1 Gene

Residual Variation Intolerance Score: 21.1% of all genes are more intolerant (likely to be disease-causing)
Gene Damage Index Score: 3.02; 50.15% of all genes are more intolerant (likely to be disease-causing)

Relevant External Links for ACOX1 Gene

Human Gene Mutation Database (HGMD)

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for ACOX1 Gene

Disorders for ACOX1 Gene

MalaCards: The human disease database

(6) MalaCards diseases for ACOX1 Gene - From: OMIM, ClinVar, GeneTests, Orphanet, Swiss-Prot, DISEASES, Novoseek, and GeneCards

Disorder Aliases PubMed IDs
peroxisomal acyl-coa oxidase deficiency
  • peroxisomal acyl-coenzyme a oxidase
  • adrenomyeloneuropathy, adult
d-bifunctional protein deficiency
  • 17-beta-hydroxysteroid dehydrogenase iv deficiency
chondrodysplasia punctata, rhizomelic, type 1
  • rhizomelic chondrodysplasia punctata, type 1
- elite association - COSMIC cancer census association via MalaCards
Search ACOX1 in MalaCards View complete list of genes associated with diseases


  • Adrenoleukodystrophy, pseudoneonatal (Pseudo-NALD) [MIM:264470]: A peroxisomal single-enzyme disorder of fatty acid beta-oxidation, resulting in clinical manifestations that remind neonatal adrenoleukodystrophy. Clinical features include mental retardation, leukodystrophy, seizures, mild hepatomegaly, hearing deficit. Pseudo-NALD is characterized by increased plasma levels of very-long chain fatty acids, due to decreased or absent peroxisome acyl-CoA oxidase activity. Peroxisomes are intact and functioning. {ECO:0000269 PubMed:11815777, ECO:0000269 PubMed:17458872, ECO:0000269 PubMed:8040306}. Note=The disease is caused by mutations affecting the gene represented in this entry.

Relevant External Links for ACOX1

Genetic Association Database (GAD)
Human Genome Epidemiology (HuGE) Navigator
Atlas of Genetics and Cytogenetics in Oncology and Haematology:
genes like me logo Genes that share disorders with ACOX1: view

No data available for Genatlas for ACOX1 Gene

Publications for ACOX1 Gene

  1. Isolation of the human peroxisomal acyl-CoA oxidase gene: organization, promoter analysis, and chromosomal localization. (PMID: 8159712) Varanasi U. … Reddy J.K. (Proc. Natl. Acad. Sci. U.S.A. 1994) 2 3 4 23 67
  2. Reversal of mouse Acyl-CoA oxidase 1 (ACOX1) null phenotype by human ACOX1b isoform. (PMID: 20195242) Vluggens A. … Cherkaoui-Malki M. (Lab. Invest. 2010) 3 23
  3. Peroxisomal acyl-CoA-oxidase deficiency: two new cases. (PMID: 18536048) Carrozzo R. … Bonioli E. (Am. J. Med. Genet. A 2008) 3 23
  4. Clinical, biochemical, and mutational spectrum of peroxisomal acyl- coenzyme A oxidase deficiency. (PMID: 17458872) Ferdinandusse S. … Waterham H.R. (Hum. Mutat. 2007) 3 23
  5. Biochemical characterization of two functional human liver acyl-CoA oxidase isoforms 1a and 1b encoded by a single gene. (PMID: 17603022) Oaxaca-Castillo D. … Cherkaoui-Malki M. (Biochem. Biophys. Res. Commun. 2007) 3 23

Products for ACOX1 Gene

Sources for ACOX1 Gene