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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ACOT2
acyl-CoA thioesterase 2
Chromosome 14 · 14q24.3
NCBI Gene: 10965Ensembl: ENSG00000119673.17HGNC: HGNC:18431UniProt: B3KSA0
40PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
acyl-CoA metabolic processprotein bindingmitochondrionfatty acyl-CoA hydrolase activitydengue diseaseHallux valgusacute myeloid leukemiaBlackfan-Diamond anemia
✦AI Summary

ACOT2 is a mitochondrial acyl-CoA thioesterase that catalyzes hydrolysis of acyl-CoAs into free fatty acids and coenzyme A, with preferential activity toward long-chain acyl-CoAs (C14-C20) 12. The enzyme contains an alpha/beta hydrolase fold with a catalytic triad (Ser294-His422-Asp388) 3, functioning as a regulator of intracellular fatty acid levels and hepatic fatty acid oxidation in mitochondria. Beyond its canonical lipase function, ACOT2 exhibits unexpected serine protease activity, cleaving dengue virus polyproteins to promote viral replication 4. Selective ACOT2 knockdown suppresses dengue serotype 2 replication, indicating its importance during viral infection 5. In hepatic metabolism, ACOT2 expression decreases in metabolic dysfunction-associated steatotic liver disease, and restoring ACOT2 levels may prevent fatty liver disease 6. Clinically, elevated ACOT2 expression in acute myeloid leukemia predicts poor overall survival and correlates with abnormal lipid metabolism 7. Genome-wide association studies identified ACOT2 as a pancreatic cancer susceptibility gene 8. ACOT2 also represents a gene regulatory hub in responses to endocrine-disrupting chemicals affecting cardiometabolic disease pathways 9, suggesting multiple therapeutic targeting opportunities.

Sources cited
1
ACOT2 catalyzes hydrolysis of acyl-CoAs into free fatty acids and coenzyme A with higher activity toward long-chain acyl-CoAs (C14-C20)
PMID: 10944470
2
ACOT2 is a mitochondrial ACOT that hydrolyzes acyl-CoAs to maintain cellular fatty acid and acyl-CoA balance
PMID: 16940157
3
ACOT2 crystal structure shows alpha/beta hydrolase fold with catalytic triad Ser294-His422-Asp388
PMID: 19497300
4
ACOT2 functions as a serine protease that hydrolyzes DENV polyproteins to assist viral replication
PMID: 38112462
5
Mitochondrial ACOT2 knockdown decreases DENV2 protein translation, genome replication, and infectious virus release
PMID: 35215835
6
ACOT2 expression is decreased in metabolic dysfunction-associated steatotic liver disease; restoring ACOT2 may prevent fatty liver disease
PMID: 40271687
7
High ACOT2 expression in acute myeloid leukemia predicts worse overall survival and abnormal lipid metabolism
PMID: 36193167
8
ACOT2 identified as a pancreatic adenocarcinoma susceptibility gene through transcriptome-wide association study
PMID: 39002386
9
ACOT2 is a gene regulatory hub and network regulator in liver responses to endocrine-disrupting chemicals affecting cardiometabolic disease
PMID: 38043319
Disease Associationsⓘ20
dengue diseaseOpen Targets
0.37Weak
Hallux valgusOpen Targets
0.13Weak
acute myeloid leukemiaOpen Targets
0.07Suggestive
Blackfan-Diamond anemiaOpen Targets
0.07Suggestive
alpha thalassemia-intellectual disability syndrome type 1Open Targets
0.06Suggestive
Alpha-thalassemia - intellectual disability syndrome linked to chromosome 16Open Targets
0.06Suggestive
Adult-onset autosomal recessive sideroblastic anemiaOpen Targets
0.06Suggestive
hemoglobin D diseaseOpen Targets
0.06Suggestive
delta-beta-thalassemiaOpen Targets
0.05Suggestive
IRIDA syndromeOpen Targets
0.05Suggestive
Hemoglobin E - beta-thalassemiaOpen Targets
0.05Suggestive
hemoglobin E-beta-thalassemia syndromeOpen Targets
0.05Suggestive
hypertriglyceridemia 2Open Targets
0.05Suggestive
Hemoglobin C - beta-thalassemiaOpen Targets
0.05Suggestive
hemoglobin C-beta-thalassemia syndromeOpen Targets
0.05Suggestive
Rh deficiency syndromeOpen Targets
0.05Suggestive
gluthathione peroxidase deficiencyOpen Targets
0.05Suggestive
hemolytic anemia due to erythrocyte adenosine deaminase overproductionOpen Targets
0.05Suggestive
PyropoikilocytosisOpen Targets
0.05Suggestive
pyropoikilocytosis, hereditaryOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ACOT8Protein interaction96%ACOT7Protein interaction90%PFKLProtein interaction90%TECRProtein interaction90%FBP1Protein interaction90%PFKMProtein interaction90%
Tissue Expression6 tissues
Liver
100%
Heart
46%
Lung
24%
Brain
19%
Ovary
15%
Bone Marrow
6%
Gene Interaction Network
Click a node to explore
ACOT2ACOT8ACOT7PFKLTECRFBP1PFKM
PROTEIN STRUCTURE
Preparing viewer…
PDB3HLK · 2.10 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.19LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.77 [0.52–1.19]
RankingsWhere ACOT2 stands among ~20K protein-coding genes
  • #10,121of 20,598
    Most Researched40
  • #12,511of 17,882
    Most Constrained (LOEUF)1.19
Genes detectedACOT2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
High Expression of ACOT2 Predicts Worse Overall Survival and Abnormal Lipid Metabolism: A Potential Target for Acute Myeloid Leukemia.
PMID: 36193167
J Healthc Eng · 2022
1.00
2
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
0.90
3
Host serine protease ACOT2 assists DENV proliferation by hydrolyzing viral polyproteins.
PMID: 38112462
mSystems · 2024
0.80
4
Transcriptome-wide association study and Mendelian randomization in pancreatic cancer identifies susceptibility genes and causal relationships with type 2 diabetes and venous thromboembolism.
PMID: 39002386
EBioMedicine · 2024
0.70
5
A host enzyme reduces metabolic dysfunction-associated steatotic liver disease (MASLD) by inactivating intestinal lipopolysaccharide.
PMID: 40271687
Elife · 2025
0.60