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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ACOT4
acyl-CoA thioesterase 4
Chromosome 14 · 14q24.3
NCBI Gene: 122970Ensembl: ENSG00000177465.5HGNC: HGNC:19748UniProt: Q8N9L9
15PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
dicarboxylic acid catabolic processperoxisomedicarboxylic acid metabolic processshort-chain fatty acid metabolic processvertebral column disorderIntellectual disabilityacute myeloid leukemiaglioblastoma multiforme
✦AI Summary

ACOT4 (acyl-CoA thioesterase 4) is a peroxisomal enzyme that catalyzes hydrolysis of acyl-CoA thioesters into free fatty acids and coenzyme A, regulating intracellular lipid levels 1. ACOT4 exhibits broad substrate specificity, functioning as a succinyl-CoA thioesterase while also hydrolyzing glutaryl-CoA and long-chain saturated acyl-CoAs 12. In humans, ACOT4 is the sole peroxisomal ACOT and functionally encompasses activities of multiple mouse peroxisomal thioesterases through convergent evolution 1. Mechanistically, ACOT4 regulation involves AKT-mediated phosphorylation at S392, which decreases binding to HSPA1A and promotes ACOT4 accumulation 3. The enzyme activates Akt/mTOR signaling pathways, protecting cells from calcium oxalate-induced apoptosis and injury 4. Clinically, ACOT4 expression is negatively correlated with pancreatic ductal carcinoma patient survival, with depletion attenuating tumor proliferation 3. ACOT4 represents part of a 10-gene fatty acid metabolism signature predictive of endometrial cancer prognosis 5. In inflammatory skin diseases, ACOT4 is differentially expressed in psoriatic sebaceous glands, contributing to disease-specific lipid metabolism alterations 6. These findings establish ACOT4 as both a metabolic regulator and potential therapeutic target across multiple disease contexts.

Sources cited
1
ACOT4 catalyzes hydrolysis of acyl-CoAs to free fatty acids and CoASH; functions as peroxisomal succinyl-CoA thioesterase active on glutaryl-CoA and long-chain acyl-CoAs; is the sole human peroxisomal ACOT incorporating functions of three mouse genes
PMID: 16940157
2
ACOT4 is a peroxisomal acyl-CoA thioesterase with specific activity on dicarboxylyl-CoA esters, particularly succinyl-CoA, and is highly expressed in kidney and liver
PMID: 16141203
3
ACOT4 expression negatively correlates with pancreatic ductal carcinoma patient survival; AKT phosphorylates ACOT4 at S392 decreasing HSPA1A binding; ACOT4 depletion attenuates PDAC proliferation and tumor formation
PMID: 33148467
4
ACOT4 overexpression reduces calcium oxalate-induced kidney cell injury; ACOT4 activates Akt/mTOR signaling and inhibits apoptosis in renal tubular cells
PMID: 40544826
5
ACOT4 is one of 10 fatty acid metabolism genes in a prognostic signature for endometrial cancer risk stratification
PMID: 37796199
6
ACOT4 is differentially expressed in psoriatic sebaceous glands and contributes to disease-specific lipid metabolism alterations
PMID: 38433843
7
Human peroxisomes contain ACOT4 and ACOT8 as ACOTs catalyzing hydrolysis of short-, medium-, long- and very long-chain acyl-CoAs as auxiliary enzymes in peroxisomal lipid oxidation
PMID: 22465940
Disease Associationsⓘ20
vertebral column disorderOpen Targets
0.29Weak
Intellectual disabilityOpen Targets
0.15Weak
acute myeloid leukemiaOpen Targets
0.03Suggestive
glioblastoma multiformeOpen Targets
0.02Suggestive
cancerOpen Targets
0.01Suggestive
non-alcoholic steatohepatitisOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
gastric carcinomaOpen Targets
0.01Suggestive
non-alcoholic fatty liver diseaseOpen Targets
0.01Suggestive
pancreatic ductal adenocarcinomaOpen Targets
0.01Suggestive
type 2 diabetes mellitusOpen Targets
0.01Suggestive
neoplasmOpen Targets
0.00Suggestive
Hepatic steatosisOpen Targets
0.00Suggestive
rosaceaOpen Targets
0.00Suggestive
non-alcoholic fatty liverOpen Targets
0.00Suggestive
diffuse large B-cell lymphomaOpen Targets
0.00Suggestive
mastitisOpen Targets
0.00Suggestive
overnutritionOpen Targets
0.00Suggestive
ulcerative colitisOpen Targets
0.00Suggestive
VitiligoOpen Targets
0.00Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
FBP1Protein interaction91%PFKLProtein interaction91%PFKMProtein interaction91%PFKPProtein interaction91%FBP2Protein interaction91%TECRProtein interaction90%
Tissue Expression6 tissues
Liver
100%
Brain
16%
Lung
7%
Bone Marrow
7%
Heart
6%
Ovary
4%
Gene Interaction Network
Click a node to explore
ACOT4FBP1PFKLPFKMPFKPFBP2TECR
PROTEIN STRUCTURE
Preparing viewer…
PDB3K2I · 2.40 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.59LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.02 [0.67–1.59]
RankingsWhere ACOT4 stands among ~20K protein-coding genes
  • #15,479of 20,598
    Most Researched15
  • #15,611of 17,882
    Most Constrained (LOEUF)1.59
Genes detectedACOT4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Spatial transcriptomics reveals altered lipid metabolism and inflammation-related gene expression of sebaceous glands in psoriasis and atopic dermatitis.
PMID: 38433843
Front Immunol · 2024
1.00
2
ACOT4 and ACOT6 Activate Akt-mTOR Pathway and Inhibit Calcium Oxalate-Induced Renal Tubular Cell Injury.
PMID: 40544826
Kidney Blood Press Res · 2025
0.90
3
PMID: 24624464
0.80
4
ACOT4 accumulation via AKT-mediated phosphorylation promotes pancreatic tumourigenesis.
PMID: 33148467
Cancer Lett · 2021
0.70
5
Analysis of the mouse and human acyl-CoA thioesterase (ACOT) gene clusters shows that convergent, functional evolution results in a reduced number of human peroxisomal ACOTs.
PMID: 16940157
FASEB J · 2006
0.60