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

Summary

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

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_000077.7 Genomic Context describing neighboring genes

Genomic regions, transcripts, and products

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Go to reference sequence details

Genomic Sequence:

Go to nucleotide: Graphics FASTA GenBank

Expression

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See details

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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GeneRIFs: Gene References Into Functions

What's a GeneRIF?

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.
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.
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.
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.
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.
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.
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.
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.
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.

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Variation

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Alleles

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

Targeted (1) 1 citation
Chemically induced (ENU) (1) 1 citation
Endonuclease-mediated (4) 1 citation

Interactions

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Products	Interactant	Other Gene	Complex	Source	Pubs	Description

General gene information

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Markers

AI042784 (e-PCR)
- UniSTS:159514

Acox1 (e-PCR), detects polymorphism
- UniSTS:164676

Acox1 (e-PCR), detects polymorphism
- UniSTS:506704

GDB:574056 (e-PCR)
- UniSTS:157791

Acox1 (e-PCR), detects polymorphism
- UniSTS:502701

GDB:340951 (e-PCR)
- UniSTS:156628

PMC199556P1 (e-PCR)
- UniSTS:271869

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 more info	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

EC 1.3.3.6

NP_001364450.1

EC 1.3.3.6

NP_001364451.1

EC 1.3.3.6

NP_056544.2

EC 1.3.3.6

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)

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
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
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
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