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9 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ABHD10
abhydrolase domain containing 10, depalmitoylase
Chromosome 3 · 3q13.2
NCBI Gene: 55347Ensembl: ENSG00000144827.10HGNC: HGNC:25656UniProt: Q9NUJ1
61PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
hydrolase activity, hydrolyzing O-glycosyl compoundsprotein bindingpalmitoyl-(protein) hydrolase activityprotein depalmitoylationatrial fibrillationlacrimal apparatus diseaseazoospermiaimmunodeficiency 88
✦AI Summary

ABHD10 is a mitochondrial acyl-protein thioesterase that functions as an S-depalmitoylase with critical roles in redox homeostasis and drug metabolism 1. Its primary enzymatic function involves hydrolyzing palmitate and other fatty acids from protein substrates, regulating the post-translational lipid modification status of target proteins 1. Mechanistically, ABHD10 catalyzes S-depalmitoylation of peroxiredoxin-5 (PRDX5), a key mitochondrial antioxidant protein, thereby modulating mitochondrial antioxidant capacity 1. Beyond redox regulation, ABHD10 catalyzes deglucuronidation of acyl-glucuronides, including mycophenolic acid acyl-glucuronide and probenecid acyl-glucuronide, functioning as a detoxification enzyme for immunosuppressant and uricosuric drug metabolites 23. Disease relevance spans multiple contexts: In alcoholic liver disease, ABHD10 downregulation via ELK-3 signaling increases PRDX5 palmitoylation, driving oxidative stress and hepatocyte dysfunction; ABHD10 overexpression ameliorates liver damage in animal models 4. Additionally, ABHD10 is essential for male fertility, with knockout mice exhibiting sperm motility defects and malformed mitochondrial sheaths due to hyper-palmitoylation of sheath formation factors 5. Clinically, ABHD10 represents a therapeutic target for hepatotoxicity and potential biomarker for cardiac conduction through associations with PR interval 6.

Sources cited
1
ABHD10 is a mitochondrial acyl-protein thioesterase and S-depalmitoylase that regulates PRDX5 depalmitoylation and mitochondrial antioxidant capacity
PMID: 31740833
2
ABHD10 catalyzes deglucuronidation of mycophenolic acid acyl-glucuronide as a detoxification enzyme
PMID: 22294686
3
ABHD10 catalyzes deglucuronidation of probenecid acyl-glucuronide and regulates its formation
PMID: 25217485
4
ABHD10 downregulation in alcoholic liver disease via ELK-3 increases PRDX5 palmitoylation and oxidative stress; ABHD10 overexpression ameliorates liver damage
PMID: 37400491
5
ABHD10 is essential for male fertility and sperm mitochondrial sheath formation by depalmitoylating SPATA19, GK2, and PDHX
PMID: 41285782
6
ABHD10 has causal associations with cardiac conduction parameters, specifically PR interval
PMID: 39486179
Disease Associationsⓘ20
atrial fibrillationOpen Targets
0.09Suggestive
lacrimal apparatus diseaseOpen Targets
0.06Suggestive
azoospermiaOpen Targets
0.06Suggestive
immunodeficiency 88Open Targets
0.05Suggestive
preeclampsiaOpen Targets
0.05Suggestive
linear and whorled nevoid hypermelanosisOpen Targets
0.05Suggestive
chronic myeloproliferative disorderOpen Targets
0.05Suggestive
external ear diseaseOpen Targets
0.05Suggestive
eosinophil peroxidase deficiencyOpen Targets
0.04Suggestive
Abnormality of the integumentOpen Targets
0.04Suggestive
neutropenia, severe congenital, 1, autosomal dominantOpen Targets
0.04Suggestive
spermatogenic failure 61Open Targets
0.04Suggestive
spermatogenic failure 62Open Targets
0.04Suggestive
spermatogenic failure 88Open Targets
0.04Suggestive
spermatogenic failure 59Open Targets
0.04Suggestive
spermatogenic failure 60Open Targets
0.04Suggestive
spermatogenic failure 73Open Targets
0.04Suggestive
spermatogenic failure 74Open Targets
0.04Suggestive
Male infertility with spermatogenesis disorder due to single gene mutationOpen Targets
0.04Suggestive
spermatogenic failure, X-linked, 5Open Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
CMBLShared pathway33%RBBP9Shared pathway33%FMO4Shared pathway33%AOX1Shared pathway33%NAT2Shared pathway33%NAT1Shared pathway33%
Tissue Expression6 tissues
Brain
100%
Heart
94%
Liver
68%
Bone Marrow
54%
Ovary
38%
Lung
20%
Gene Interaction Network
Click a node to explore
ABHD10CMBLRBBP9FMO4AOX1NAT2NAT1
PROTEIN STRUCTURE
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AlphaFoldAI-predicted · UniProt Q9NUJ1
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.04LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.71 [0.50–1.04]
RankingsWhere ABHD10 stands among ~20K protein-coding genes
  • #7,530of 20,598
    Most Researched61
  • #10,314of 17,882
    Most Constrained (LOEUF)1.04
Genes detectedABHD10
Sources retrieved9 papers
Response time—
📄 Sources
9▼
1
The Elk-3 target Abhd10 ameliorates hepatotoxic injury and fibrosis in alcoholic liver disease.
PMID: 37400491
Commun Biol · 2023
1.00
2
ABHD10 is an S-depalmitoylase affecting redox homeostasis through peroxiredoxin-5.
PMID: 31740833
Nat Chem Biol · 2019
0.89
3
The emerging role of human esterases.
PMID: 22813719
Drug Metab Pharmacokinet · 2012
0.78
4
The S-depalmitoylase ABHD10 is essential for sperm mitochondrial sheath formation and male fertility.
PMID: 41285782
Nat Commun · 2025
0.67
5
[Role of Human Orphan Esterases in Drug-induced Toxicity].
PMID: 26521872
Yakugaku Zasshi · 2015
0.56