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Acox1 acyl-Coenzyme A oxidase 1, palmitoyl [ Mus musculus (house mouse) ]

Gene ID: 11430, updated on 5-Mar-2024

Summary

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Official Symbol
Acox1provided by MGI
Official Full Name
acyl-Coenzyme A oxidase 1, palmitoylprovided by MGI
Primary source
MGI:MGI:1330812
See related
Ensembl:ENSMUSG00000020777 AllianceGenome:MGI:1330812
Gene type
protein coding
RefSeq status
REVIEWED
Organism
Mus musculus
Lineage
Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Mus; Mus
Also known as
AOX; Acox; Paox; D130055E20Rik
Summary
This gene encodes a member of the acyl-coenzyme A oxidase family. The encoded protein is localized to peroxisomes and is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-coenzyme A to 2-trans-enoyl-coenzyme A. Disruption of this gene results in microvesicular steatohepatitis, spontaneous peroxisome proliferation, and the eventual development of hepatocellular carcinomas. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2012]
Expression
Broad expression in liver adult (RPKM 251.9), liver E18 (RPKM 159.5) and 21 other tissues See more
Orthologs
human all
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Genomic context

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Location:
11 E2; 11 80.96 cM
Exon count:
15
Annotation release Status Assembly Chr Location
RS_2024_02 current GRCm39 (GCF_000001635.27) 11 NC_000077.7 (116062714..116089605, complement)
108.20200622 previous assembly GRCm38.p6 (GCF_000001635.26) 11 NC_000077.6 (116171883..116199045, complement)

Chromosome 11 - NC_000077.7Genomic Context describing neighboring genes Neighboring gene STARR-positive B cell enhancer ABC_E4017 Neighboring gene mitochondrial ribosomal protein L38 Neighboring gene STARR-seq mESC enhancer starr_31194 Neighboring gene Fas binding factor 1 Neighboring gene predicted gene, 26413 Neighboring gene STARR-seq mESC enhancer starr_31195 Neighboring gene TEN1 telomerase capping complex subunit Neighboring gene cyclin dependent kinase 3

Genomic regions, transcripts, and products

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Go to nucleotide: Graphics FASTA GenBank

Expression

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  • Project title: Mouse ENCODE transcriptome data
  • Description: RNA profiling data sets generated by the Mouse ENCODE project.
  • BioProject: PRJNA66167
  • Publication: PMID 25409824
  • Analysis date: n/a

Bibliography

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Related articles in PubMed

  1. A microglial cell model for acyl-CoA oxidase 1 deficiency. Raas Q, et al. Biochim Biophys Acta Mol Cell Biol Lipids, 2019 Apr. PMID 30312667
  2. Hepatic Metabolic, Inflammatory, and Stress-Related Gene Expression in Growing Mice Consuming a Low Dose of Trans-10, cis-12-Conjugated Linoleic Acid. Li J, et al. J Lipids, 2012. PMID 22988513, Free PMC Article
  3. Lampe1: an ENU-germline mutation causing spontaneous hepatosteatosis identified through targeted exon-enrichment and next-generation sequencing. Sheridan R, et al. PLoS One, 2011. PMID 21760938, Free PMC Article
  4. Application of comparative functional genomics to identify best-fit mouse models to study human cancer. Lee JS, et al. Nat Genet, 2004 Dec. PMID 15565109
  5. 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. Meyer K, et al. Carcinogenesis, 2003 May. PMID 12771043

See all (75) citations in PubMed

GeneRIFs: Gene References Into Functions

What's a GeneRIF?
  1. Under peroxisome proliferation acyl-CoA oxidase coordinates with catalase to enhance ethanol metabolism.
    Title: Under peroxisome proliferation acyl-CoA oxidase coordinates with catalase to enhance ethanol metabolism.
  2. AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.
    Title: AOX delays the onset of the lethal phenotype in a mouse model of Uqcrh (complex III) disease.
  3. MiR-103-3p promotes hepatic steatosis to aggravate nonalcoholic fatty liver disease by targeting of ACOX1.
    Title: MiR-103-3p promotes hepatic steatosis to aggravate nonalcoholic fatty liver disease by targeting of ACOX1.
  4. In non-alcoholic fatty liver disease miR-222 promotes the accumulation of triglycerides by inhibiting ACOX1.
    Title: miR-222 targets ACOX1, promotes triglyceride accumulation in hepatocytes.
  5. Study describes an Acox1-deficient cell line presenting the main biochemical characteristics of severe peroxisomal leukodystrophy and will serve as a promising model to further investigate the consequences of a specific microglial peroxisomal beta-oxidation defect on oxidative stress, inflammation and cellular functions.
    Title: A microglial cell model for acyl-CoA oxidase 1 deficiency.
  6. Our data support a link between oxidative stress and the deficiency of Abcd1 or Acox1 peroxisomal proteins.
    Title: 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.
  7. progressive PPARalpha- and p8-mediated ER stress contribute to the hepatocarcinogenesis in ACOX1(-/-) mice
    Title: Progressive endoplasmic reticulum stress contributes to hepatocarcinogenesis in fatty acyl-CoA oxidase 1-deficient mice.
  8. Loss of Fatty acyl-CoA oxidase leads to increased amounts of PPARalpha, peroxisomal bifunctional enzyme and thiolase in hepatocellular carcinoma
    Title: Expression of peroxisome proliferator-activated receptor alpha, and PPARalpha regulated genes in spontaneously developed hepatocellular carcinomas in fatty acyl-CoA oxidase null mice.
  9. STAT5A binds to -1841 to -1825 of the murine AOX promoter.
    Title: STAT5 activators modulate acyl CoA oxidase (AOX) expression in adipocytes and STAT5A binds to the AOX promoter in vitro.
Submit: New GeneRIF Correction

Variation

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Alleles

Alleles of this type are documented at Mouse Genome Informatics  (MGI)

Pathways from PubChem

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Interactions

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Products Interactant Other Gene Complex Source Pubs Description

General gene information

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Markers

Homology

Clone Names

  • D130055E20Rik

Gene Ontology Provided by MGI

Function Evidence Code Pubs
enables FAD binding ISO
Inferred from Sequence Orthology
more info
  
enables PDZ domain binding ISO
Inferred from Sequence Orthology
more info
 PubMed 
enables acyl-CoA oxidase activity ISO
Inferred from Sequence Orthology
more info
  
enables acyl-CoA oxidase activity TAS
Traceable Author Statement
more info
 PubMed 
enables fatty acid binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables fatty acid binding ISO
Inferred from Sequence Orthology
more info
  
enables flavin adenine dinucleotide binding IBA
Inferred from Biological aspect of Ancestor
more info
  
enables flavin adenine dinucleotide binding ISO
Inferred from Sequence Orthology
more info
  
enables oxidoreductase activity IEA
Inferred from Electronic Annotation
more info
  
enables oxidoreductase activity, acting on the CH-CH group of donors IEA
Inferred from Electronic Annotation
more info
  
enables palmitoyl-CoA oxidase activity IBA
Inferred from Biological aspect of Ancestor
more info
  
enables palmitoyl-CoA oxidase activity ISO
Inferred from Sequence Orthology
more info
  
enables protein homodimerization activity ISO
Inferred from Sequence Orthology
more info
  
Process Evidence Code Pubs
involved_in cholesterol homeostasis ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within fatty acid beta-oxidation TAS
Traceable Author Statement
more info
 PubMed 
involved_in fatty acid beta-oxidation using acyl-CoA oxidase IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in fatty acid beta-oxidation using acyl-CoA oxidase ISO
Inferred from Sequence Orthology
more info
  
involved_in fatty acid catabolic process ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within fatty acid metabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in fatty acid metabolic process IEA
Inferred from Electronic Annotation
more info
  
involved_in fatty acid oxidation IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in fatty acid oxidation ISO
Inferred from Sequence Orthology
more info
  
involved_in generation of precursor metabolites and energy ISO
Inferred from Sequence Orthology
more info
  
involved_in hydrogen peroxide biosynthetic process ISO
Inferred from Sequence Orthology
more info
  
involved_in lipid homeostasis IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in lipid homeostasis ISO
Inferred from Sequence Orthology
more info
  
involved_in lipid metabolic process ISO
Inferred from Sequence Orthology
more info
  
involved_in peroxisome fission ISO
Inferred from Sequence Orthology
more info
  
involved_in prostaglandin metabolic process ISO
Inferred from Sequence Orthology
more info
  
acts_upstream_of_or_within spermatogenesis IMP
Inferred from Mutant Phenotype
more info
 PubMed 
involved_in very long-chain fatty acid beta-oxidation ISO
Inferred from Sequence Orthology
more info
  
involved_in very long-chain fatty acid metabolic process IBA
Inferred from Biological aspect of Ancestor
more info
  
involved_in very long-chain fatty acid metabolic process ISO
Inferred from Sequence Orthology
more info
  
Component Evidence Code Pubs
located_in cytoplasm IDA
Inferred from Direct Assay
more info
 PubMed 
located_in cytosol TAS
Traceable Author Statement
more info
  
located_in mitochondrion HDA PubMed 
located_in peroxisomal membrane IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in peroxisome IBA
Inferred from Biological aspect of Ancestor
more info
  
located_in peroxisome IDA
Inferred from Direct Assay
more info
 PubMed 
is_active_in peroxisome ISO
Inferred from Sequence Orthology
more info
  
located_in peroxisome ISO
Inferred from Sequence Orthology
more info
  
located_in peroxisome TAS
Traceable Author Statement
more info
 PubMed 

General protein information

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Preferred Names
peroxisomal acyl-coenzyme A oxidase 1
Names
palmitoyl-CoA oxidase
peroxisomal fatty acyl-CoA oxidase
peroxisomal fatty acyl-coenzyme A oxidase
peroxisomal straight-chain Acyl-CoA oxidase
straight-chain acyl-CoA oxidase
NP_001258827.1
NP_001364450.1
NP_001364451.1
NP_056544.2

NCBI Reference Sequences (RefSeq)

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RefSeqs maintained independently of Annotated Genomes

These reference sequences exist independently of genome builds. Explain

These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

mRNA and Protein(s)

  1. NM_001271898.2 → NP_001258827.1  peroxisomal acyl-coenzyme A oxidase 1 isoform 2

    See identical proteins and their annotated locations for NP_001258827.1

    Status: REVIEWED

    Source sequence(s)
    AL607108, AL669925
    Consensus CDS
    CCDS70354.1
    UniProtKB/Swiss-Prot
    A2A850, O35616, Q3TDG0, Q8BYC3, Q9R0H0
    UniProtKB/TrEMBL
    A2A848
    Related
    ENSMUSP00000072717.5, ENSMUST00000072948.11
    Conserved Domains (1) summary
    cd01150
    Location:3 → 638
    AXO; Peroxisomal acyl-CoA oxidase

  2. NM_001377521.1 → NP_001364450.1  peroxisomal acyl-coenzyme A oxidase 1 isoform 3

    Status: REVIEWED

    Source sequence(s)
    AL607108, AL669925
    UniProtKB/TrEMBL
    A2A848
    Related
    ENSMUSP00000122185.2, ENSMUST00000148601.2
    Conserved Domains (1) summary
    cd01150
    Location:23 → 570
    AXO; Peroxisomal acyl-CoA oxidase

  3. NM_001377522.1 → NP_001364451.1  peroxisomal acyl-coenzyme A oxidase 1 isoform 4

    Status: REVIEWED

    Source sequence(s)
    AL607108, AL669925
    Conserved Domains (1) summary
    cl09933
    Location:3 → 562
    ACAD; Acyl-CoA dehydrogenase

  4. NM_015729.4 → NP_056544.2  peroxisomal acyl-coenzyme A oxidase 1 isoform 1

    See identical proteins and their annotated locations for NP_056544.2

    Status: REVIEWED

    Source sequence(s)
    AL607108, AL669925
    Consensus CDS
    CCDS25660.1
    UniProtKB/TrEMBL
    A2A848
    Related
    ENSMUSP00000063325.6, ENSMUST00000066587.12
    Conserved Domains (2) summary
    cd01150
    Location:3 → 638
    AXO; Peroxisomal acyl-CoA oxidase
    PLN02443
    Location:5 → 657
    PLN02443; acyl-coenzyme A oxidase

RefSeqs of Annotated Genomes: GCF_000001635.27-RS_2024_02

The following sections contain reference sequences that belong to a specific genome build. Explain

Reference GRCm39 C57BL/6J

Genomic

  1. NC_000077.7 Reference GRCm39 C57BL/6J

    Range
    116062714..116089605 complement
    Download
    GenBank, FASTA, Sequence Viewer (Graphics)
Nucleotide Protein Strain
Heading Accession and Version
Protein Accession Links
GenPept Link UniProtKB Link
Q9R0H0.5 GenPept UniProtKB/Swiss-Prot:Q9R0H0

Gene LinkOut

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