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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ACOX3
acyl-CoA oxidase 3, pristanoyl
Chromosome 4 · 4p16.1
NCBI Gene: 8310Ensembl: ENSG00000087008.17HGNC: HGNC:121UniProt: O15254
29PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
membraneperoxisomeflavin adenine dinucleotide bindingfatty acid bindingglaucomamouth diseasePathologic fracturehemorrhoid
✦AI Summary

ACOX3 (acyl-CoA oxidase 3, pristanoyl) is a peroxisomal enzyme that catalyzes the first step of fatty acid β-oxidation, specifically oxidizing CoA-esters of 2-methyl-branched fatty acids 1. The enzyme localizes to both peroxisomal matrix and cytosol, where it functions in peroxisomal fatty acid metabolism alongside other acyl-CoA oxidases (ACOX1 and ACOX2) 2. ACOX3 plays a critical role in maintaining vascular smooth muscle tone, as demonstrated by functional studies showing that ACOX3 dysfunction leads to loss of basal myogenic tone in arterial smooth muscle cells and hypersensitivity to sympathomimetic stimuli 3. The enzyme shows differential expression patterns across various cancer types, with upregulation in some cancers and downregulation in others, suggesting context-dependent roles in tumorigenesis 4. ACOX3 expression is modulated by metabolic regulators like Sirt1, particularly in cardiac lipid metabolism, where its downregulation contributes to improved cardiac function in diabetic cardiomyopathy 1. Clinical studies have identified ACOX3 as a potential therapeutic target for post-traumatic stress disorder and various cancers 56. The enzyme's activity is sensitive to pharmacological interventions, with compounds like nintedanib and atorvastatin affecting its expression in cancer and metabolic contexts 78.

Sources cited
1
ACOX3 participates in fatty acid β-oxidation and is modulated by Sirt1 in cardiac metabolism
PMID: 37957697
2
ACOX3 dysfunction causes loss of arterial smooth muscle tone and contributes to recurrent vasospasm
PMID: 31975215
3
ACOX3 shows differential expression across cancer types and serves as a pan-cancer biomarker
PMID: 41411722
4
ACOX3 functions alongside ACOX1 and ACOX2 in peroxisomal fatty acid processing
PMID: 35999530
5
ACOX3 identified as potential therapeutic target for PTSD through multi-omics analysis
PMID: 41378860
6
ACOX3 serves as prognostic biomarker in osteosarcoma through lipid metabolism-acetylation pathway
PMID: 39162397
7
ACOX3 expression affected by nintedanib treatment in gastric cancer cells
PMID: 38406279
8
ACOX3 expression modulated by atorvastatin in fish hypolipidemic model
PMID: 38535952
Disease Associationsⓘ20
glaucomaOpen Targets
0.32Weak
mouth diseaseOpen Targets
0.28Weak
Pathologic fractureOpen Targets
0.27Weak
hemorrhoidOpen Targets
0.26Weak
skin woundOpen Targets
0.26Weak
atrial fibrillationOpen Targets
0.25Weak
lymphatic system diseaseOpen Targets
0.09Suggestive
vein disorderOpen Targets
0.09Suggestive
Abruptio PlacentaeOpen Targets
0.08Suggestive
early-onset non-syndromic cataractOpen Targets
0.06Suggestive
Total congenital cataractOpen Targets
0.05Suggestive
Cataract-microcornea syndromeOpen Targets
0.05Suggestive
Cataract with Y-shaped suture opacitiesOpen Targets
0.05Suggestive
HypoxemiaOpen Targets
0.05Suggestive
hereditary hyperferritinemia with congenital cataractsOpen Targets
0.05Suggestive
poisoningOpen Targets
0.05Suggestive
asphyxiaOpen Targets
0.05Suggestive
Posterior polar cataractOpen Targets
0.05Suggestive
Partial congenital cataractOpen Targets
0.05Suggestive
Familial ocular anterior segment mesenchymal dysgenesisOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ACOXLShared pathway100%PEX2Protein interaction100%ACAA2Protein interaction100%ACADLProtein interaction99%ACADSBProtein interaction99%ACADVLProtein interaction99%
Tissue Expression6 tissues
Liver
100%
Heart
75%
Lung
52%
Ovary
52%
Brain
38%
Bone Marrow
29%
Gene Interaction Network
Click a node to explore
ACOX3ACOXLPEX2ACAA2ACADLACADSBACADVL
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt O15254
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.11LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.92 [0.76–1.11]
RankingsWhere ACOX3 stands among ~20K protein-coding genes
  • #12,085of 20,598
    Most Researched29
  • #11,439of 17,882
    Most Constrained (LOEUF)1.11
Genes detectedACOX3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Treatment with recombinant Sirt1 rewires the cardiac lipidome and rescues diabetes-related metabolic cardiomyopathy.
PMID: 37957697
Cardiovasc Diabetol · 2023
1.00
2
ACOX3 Dysfunction as a Potential Cause of Recurrent Spontaneous Vasospasm of Internal Carotid Artery.
PMID: 31975215
Transl Stroke Res · 2020
0.90
3
Multi-omics profiling of ACOX3 unveils pan-cancer clinical biomarker potential.
PMID: 41411722
Comput Biol Chem · 2026
0.80
4
Integrating multi-omics to reveal the causal effect of gut microbiota on post-traumatic stress disorder and potential drug target analysis.
PMID: 41378860
Eur J Psychotraumatol · 2025
0.70
5
Proteomic study on nintedanib in gastric cancer cells.
PMID: 38406279
PeerJ · 2024
0.60