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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ABHD11
abhydrolase domain containing 11
Chromosome 7 · 7q11.23
NCBI Gene: 83451Ensembl: ENSG00000106077.19HGNC: HGNC:16407UniProt: C9J7Q4
34PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
mitochondrionlipase activitymitochondrial matrixoxoglutarate dehydrogenase complexneurodegenerative diseasecolorectal carcinomapapillary thyroid carcinomalung cancer
✦AI Summary

ABHD11 encodes a mitochondrial α/β-hydrolase domain-containing protein that serves multiple critical functions in cellular metabolism. The protein maintains the catalytic function of the α-ketoglutarate dehydrogenase (α-KGDH) complex in the TCA cycle by removing glutaryl adducts from lipoate, an essential fatty acid modification required for TCA cycle function 1. This deglutarylation activity prevents the accumulation of glutaryl-lipoyl adducts that would otherwise compromise mitochondrial metabolism 1. ABHD11 also functions as a lipid metabolism regulator, catalyzing diacylglycerol hydrolysis with substrate preference for 1,2-diacyl-sn-glycerols over 1,3-diacyl-sn-glycerols. In T-cell biology, ABHD11 plays a crucial role in immune regulation. Pharmacological inhibition of ABHD11 reduces cytokine production in human and mouse T-cells by increasing 24,25-epoxycholesterol biosynthesis and activating liver X receptor (LXR), which arises from TCA cycle compromise 23. This anti-inflammatory effect has therapeutic potential, as ABHD11 inhibition delays diabetes onset in murine autoimmune models 3. The protein's expression in CD4+ T-cells correlates with remission status in rheumatoid arthritis, highlighting its clinical relevance in autoimmune diseases 23.

Sources cited
1
ABHD11 removes glutaryl adducts from lipoate to maintain TCA cycle function and prevent glutaryl-lipoyl adduct accumulation
PMID: 40664791
2
ABHD11 inhibition dampens T-cell cytokine production through increased 24,25-epoxycholesterol biosynthesis and LXR activation
PMID: 41184294
3
ABHD11 inhibition has anti-inflammatory effects and delays diabetes onset in autoimmune models, with expression linked to rheumatoid arthritis remission status
PMID: 40166327
⚠Limited data available — This gene has 3 indexed publications. Summary and analysis may be incomplete.
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.46Moderate
colorectal carcinomaOpen Targets
0.10Weak
papillary thyroid carcinomaOpen Targets
0.10Suggestive
lung cancerOpen Targets
0.07Suggestive
ovarian cancerOpen Targets
0.07Suggestive
urinary bladder carcinomaOpen Targets
0.07Suggestive
lung adenocarcinomaOpen Targets
0.06Suggestive
Abruptio PlacentaeOpen Targets
0.06Suggestive
retinitis pigmentosaOpen Targets
0.06Suggestive
Cone rod dystrophyOpen Targets
0.06Suggestive
triple-negative breast cancerOpen Targets
0.06Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.05Suggestive
breast cancerOpen Targets
0.05Suggestive
cancerOpen Targets
0.05Suggestive
Bietti crystalline dystrophyOpen Targets
0.05Suggestive
pachyonychia congenitaOpen Targets
0.05Suggestive
late-onset retinal degenerationOpen Targets
0.04Suggestive
central areolar choroidal dystrophyOpen Targets
0.04Suggestive
isolated aniridiaOpen Targets
0.04Suggestive
MORM syndromeOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
ZNF202Shared pathway100%FAM135BShared pathway100%PLCXD1Shared pathway100%FAM135AShared pathway100%PLCXD3Shared pathway100%PLCXD2Shared pathway100%
Tissue Expression6 tissues
Heart
100%
Liver
71%
Lung
58%
Brain
36%
Bone Marrow
33%
Ovary
24%
Gene Interaction Network
Click a node to explore
ABHD11ZNF202FAM135BPLCXD1FAM135APLCXD3PLCXD2
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q8NFV4
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.19LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.63–1.19]
RankingsWhere ABHD11 stands among ~20K protein-coding genes
  • #11,103of 20,598
    Most Researched34
  • #12,537of 17,882
    Most Constrained (LOEUF)1.19
Genes detectedABHD11
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
m
PMID: 38838765
Cancer Lett · 2024
1.00
2
Mitochondrial ABHD11 inhibition drives sterol metabolism to modulate T-cell effector function.
PMID: 41184294
Nat Commun · 2025
0.90
3
ABHD11-AS1: An Emerging Long Non-Coding RNA (lncRNA) with Clinical Significance in Human Malignancies.
PMID: 35314614
Noncoding RNA · 2022
0.80
4
Lipoyl deglutarylation by ABHD11 regulates mitochondrial and T cell metabolism.
PMID: 40664791
Nat Chem Biol · 2025
0.70
5
LncRNA ABHD11-AS1 promotes tumor progression in papillary thyroid carcinoma by regulating EPS15L1/EGFR signaling pathway.
PMID: 35098448
Clin Transl Oncol · 2022
0.60