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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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OTUD3
OTU deubiquitinase 3
Chromosome 1 · 1p36.13
NCBI Gene: 23252Ensembl: ENSG00000169914.7HGNC: HGNC:29038UniProt: Q5T2D3
48PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein K11-linked deubiquitinationprotein K6-linked deubiquitinationprotein stabilizationnegative regulation of phosphatidylinositol 3-kinase/protein kinase B signal transductionulcerative colitisinflammatory bowel diseaseulcerative proctosigmoiditisintestinal disease
✦AI Summary

OTUD3 is a deubiquitinase that hydrolyzes Lys-6- and Lys-11-linked polyubiquitin chains, including heterotypic and homotypic variants 123. It functions as a metabolic regulator: glucose and fatty acids trigger OTUD3's nuclear translocation through CBP-dependent acetylation, whereupon it deubiquitinates and stabilizes the nuclear receptor PPARD to regulate genes involved in glucose metabolism, lipid metabolism, and oxidative phosphorylation 3. Additionally, OTUD3 negatively regulates ribosome quality control by deubiquitinating 40S ribosomal proteins RPS10 and RPS20, antagonizing ZNF598-mediated ubiquitination 2. Clinically, OTUD3 exhibits context-dependent disease relevance. It functions protectively in ulcerative colitis by deubiquitinating STING in intestinal fibroblasts, suppressing microbiota-mediated inflammation 4, and protects against esophageal cancer by stabilizing ZFP36, which promotes VEGF-C mRNA decay 5. Conversely, OTUD3 is oncogenic in colorectal cancer (promoting YY1 stability), renal cell carcinoma (stabilizing SLC7A11 to suppress ferroptosis), and diffuse large B-cell lymphoma (deubiquitinating PD-L1 and MYL12A) 678. OTUD3 genetic variants associate with type 2 diabetes risk and diabetic retinopathy 9, positioning OTUD3 as both a disease biomarker and potential therapeutic target 1011.

Sources cited
1
OTUD3 is a deubiquitinase that hydrolyzes Lys-6- and Lys-11-linked polyubiquitin chains, including heterotypic and homotypic variants , , .
PMID: 35675826
2
OTUD3 is a deubiquitinase that hydrolyzes Lys-6- and Lys-11-linked polyubiquitin chains, including heterotypic and homotypic variants , , .
PMID: 32011234
3
It functions protectively in ulcerative colitis by deubiquitinating STING in intestinal fibroblasts, suppressing microbiota-mediated inflammation , and protects against esophageal cancer by stabilizing ZFP36, which promotes VEGF-C mRNA decay .
PMID: 40680146
4
It functions protectively in ulcerative colitis by deubiquitinating STING in intestinal fibroblasts, suppressing microbiota-mediated inflammation , and protects against esophageal cancer by stabilizing ZFP36, which promotes VEGF-C mRNA decay .
PMID: 34853315
5
OTUD3 genetic variants associate with type 2 diabetes risk and diabetic retinopathy , positioning OTUD3 as both a disease biomarker and potential therapeutic target , .
PMID: 36531510
Disease Associationsⓘ20
ulcerative colitisOpen Targets
0.54Moderate
inflammatory bowel diseaseOpen Targets
0.50Moderate
ulcerative proctosigmoiditisOpen Targets
0.34Weak
intestinal diseaseOpen Targets
0.33Weak
rheumatoid arthritisOpen Targets
0.31Weak
ulcer diseaseOpen Targets
0.23Weak
proctitisOpen Targets
0.22Weak
breast cancerOpen Targets
0.10Suggestive
neoplasmOpen Targets
0.09Suggestive
lung cancerOpen Targets
0.09Suggestive
hepatocellular carcinomaOpen Targets
0.09Suggestive
esophageal cancerOpen Targets
0.08Suggestive
colitisOpen Targets
0.08Suggestive
colorectal carcinomaOpen Targets
0.08Suggestive
diffuse large B-cell lymphomaOpen Targets
0.07Suggestive
Parkinson diseaseOpen Targets
0.07Suggestive
enteritisOpen Targets
0.06Suggestive
ankylosing spondylitisOpen Targets
0.06Suggestive
psoriasisOpen Targets
0.06Suggestive
sclerosing cholangitisOpen Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MRPS6Protein interaction100%MRPS9Protein interaction100%MRPL36Protein interaction100%MRPS16Protein interaction100%MRPS12Protein interaction100%MRPS5Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
59%
Brain
50%
Lung
39%
Liver
26%
Heart
26%
Gene Interaction Network
Click a node to explore
OTUD3MRPS6MRPS9MRPL36MRPS16MRPS12MRPS5
PROTEIN STRUCTURE
Preparing viewer…
PDB4BOU · 1.55 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.84LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.55 [0.37–0.84]
RankingsWhere OTUD3 stands among ~20K protein-coding genes
  • #9,107of 20,598
    Most Researched48
  • #7,172of 17,882
    Most Constrained (LOEUF)0.84
Genes detectedOTUD3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
OTUD3 prevents ulcerative colitis by inhibiting microbiota-mediated STING activation.
PMID: 40680146
Sci Immunol · 2025
1.00
2
Research progress in deubiquitinase OTUD3.
PMID: 39788523
Zhong Nan Da Xue Xue Bao Yi Xue Ban · 2024
0.90
3
Phosphorylation-dependent deubiquitinase OTUD3 regulates YY1 stability and promotes colorectal cancer progression.
PMID: 38351178
Cell Death Dis · 2024
0.80
4
Nicotine-mediated OTUD3 downregulation inhibits VEGF-C mRNA decay to promote lymphatic metastasis of human esophageal cancer.
PMID: 34853315
Nat Commun · 2021
0.70
5
OTUD3-mediated stabilization of SLC7A11 drives sunitinib resistance by suppressing ferroptosis in clear cell renal cell carcinoma.
PMID: 40716486
Cancer Lett · 2025
0.60