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25 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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USP11
ubiquitin specific peptidase 11
Chromosome X · Xp11.3
NCBI Gene: 8237Ensembl: ENSG00000102226.10HGNC: HGNC:12609UniProt: G5E9A6
205PubMed Papers
20Diseases
0Drugs
2Pathogenic Variants
FUNCTIONAL ROLE
Highly Constrained
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolcysteine-type endopeptidase activitycysteine-type deubiquitinase activityprotein bindingbronchiectasisAbsent inner and outer dynein armsAbnormal brain morphologyDynein arm defect of respiratory motile cilia
✦AI Summary

USP11 is a cysteine-type deubiquitinase localized to the cytosol and nucleus that regulates protein stability through selective deubiquitination of target substrates 1. Mechanistically, USP11 removes K48-linked and K63-linked polyubiquitin chains from diverse substrates including Sirt3, AR, c-Myc, SREBF1, Nrf2, HOXC5, PRDX2, and VEGFR2, stabilizing these proteins and promoting their nuclear translocation or transcriptional activity 12345. USP11 displays context-dependent pathological roles across multiple disease states. In neurodegenerative disease, USP11 escapes X-inactivation and is elevated in females, where it promotes tau deubiquitination and acetylation, increasing tauopathy vulnerability and Alzheimer's disease risk in women 6. In cancers, USP11 overexpression drives oncogenic programs: in prostate cancer via AR/c-Myc upregulation 2, in hepatocellular carcinoma through SREBF1-mediated lipogenesis 3, in colorectal cancer via USP11/Nrf2 antioxidant feedback loops 4, and in bladder cancer through MYC stabilization and glycolysis 7. USP11 also promotes angiogenesis via PRDX2/c-MYC axis activation 5. Conversely, USP11 deletion protects against intervertebral disc degeneration by reducing ferroptosis 1 and supports spermatogenesis through HOXC5/WNT signaling 8. These findings position USP11 as a multifunctional deubiquitinase with therapeutic potential as both a target for cancer and neurodegeneration and a candidate for regenerative medicine applications.

Sources cited
1
USP11 is a cysteine-type deubiquitinase localized to the cytosol and nucleus that regulates protein stability through selective deubiquitination of target substrates .
PMID: 37099926
2
In neurodegenerative disease, USP11 escapes X-inactivation and is elevated in females, where it promotes tau deubiquitination and acetylation, increasing tauopathy vulnerability and Alzheimer's disease risk in women .
PMID: 36198316
3
Mechanistically, USP11 removes K48-linked and K63-linked polyubiquitin chains from diverse substrates including Sirt3, AR, c-Myc, SREBF1, Nrf2, HOXC5, PRDX2, and VEGFR2, stabilizing these proteins and promoting their nuclear translocation or transcriptional activity , , , , .
PMID: 39052835
4
Mechanistically, USP11 removes K48-linked and K63-linked polyubiquitin chains from diverse substrates including Sirt3, AR, c-Myc, SREBF1, Nrf2, HOXC5, PRDX2, and VEGFR2, stabilizing these proteins and promoting their nuclear translocation or transcriptional activity , , , , .
PMID: 39558331
5
Mechanistically, USP11 removes K48-linked and K63-linked polyubiquitin chains from diverse substrates including Sirt3, AR, c-Myc, SREBF1, Nrf2, HOXC5, PRDX2, and VEGFR2, stabilizing these proteins and promoting their nuclear translocation or transcriptional activity , , , , .
PMID: 39617751
6
In cancers, USP11 overexpression drives oncogenic programs: in prostate cancer via AR/c-Myc upregulation , in hepatocellular carcinoma through SREBF1-mediated lipogenesis , in colorectal cancer via USP11/Nrf2 antioxidant feedback loops , and in bladder cancer through MYC stabilization and glycolysis .
PMID: 39990228
7
Mechanistically, USP11 removes K48-linked and K63-linked polyubiquitin chains from diverse substrates including Sirt3, AR, c-Myc, SREBF1, Nrf2, HOXC5, PRDX2, and VEGFR2, stabilizing these proteins and promoting their nuclear translocation or transcriptional activity , , , , .
PMID: 40199774
8
Conversely, USP11 deletion protects against intervertebral disc degeneration by reducing ferroptosis and supports spermatogenesis through HOXC5/WNT signaling .
PMID: 38722330
Disease Associationsⓘ20
bronchiectasisOpen Targets
0.27Weak
Abnormal brain morphologyOpen Targets
0.27Weak
Abnormal ciliary motilityOpen Targets
0.27Weak
Absent inner and outer dynein armsOpen Targets
0.27Weak
Dynein arm defect of respiratory motile ciliaOpen Targets
0.27Weak
neoplasmOpen Targets
0.11Weak
breast cancerOpen Targets
0.11Weak
colorectal carcinomaOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
cancerOpen Targets
0.09Suggestive
Blackfan-Diamond anemiaOpen Targets
0.08Suggestive
prostate cancerOpen Targets
0.08Suggestive
glioblastoma multiformeOpen Targets
0.08Suggestive
hereditary persistence of fetal hemoglobin-sickle cell disease syndromeOpen Targets
0.08Suggestive
Hereditary persistence of fetal hemoglobin - beta-thalassemiaOpen Targets
0.08Suggestive
diffuse large B-cell lymphomaOpen Targets
0.08Suggestive
acute lymphoblastic leukemiaOpen Targets
0.08Suggestive
acute myeloid leukemiaOpen Targets
0.08Suggestive
melanomaOpen Targets
0.08Suggestive
posterior cortical atrophyOpen Targets
0.07Suggestive
Pathogenic Variants2
NC_000023.11:g.47240362G>ALikely pathogenic
Abnormal brain morphology
★☆☆☆
NC_000023.11:g.47242504G>ALikely pathogenic
Dynein arm defect of respiratory motile cilia;Absent inner and outer dynein arms;Abnormal ciliary motility;Bronchiectasis
★☆☆☆
View on ClinVar ↗
Related Genes
USP17L23Shared pathway100%USP54Shared pathway100%JOSD2Shared pathway100%USP31Shared pathway100%OTULINLShared pathway100%JOSD1Shared pathway100%
Tissue Expression6 tissues
Brain
100%
Ovary
59%
Heart
31%
Bone Marrow
19%
Lung
19%
Liver
10%
Gene Interaction Network
Click a node to explore
USP11USP17L23USP54JOSD2USP31OTULINLJOSD1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt G5E9A6
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.14Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.07 [0.04–0.14]
RankingsWhere USP11 stands among ~20K protein-coding genes
  • #2,053of 20,598
    Most Researched205 · top 10%
  • #4,244of 5,498
    Most Pathogenic Variants2
  • #187of 17,882
    Most Constrained (LOEUF)0.14 · top 5%
Genes detectedUSP11
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
The deubiquitinase USP11 ameliorates intervertebral disc degeneration by regulating oxidative stress-induced ferroptosis via deubiquitinating and stabilizing Sirt3.
PMID: 37099926
Redox Biol · 2023
1.00
2
X-linked ubiquitin-specific peptidase 11 increases tauopathy vulnerability in women.
PMID: 36198316
Cell · 2022
0.90
3
USP11 promotes prostate cancer progression by up-regulating AR and c-Myc activity.
PMID: 39052835
Proc Natl Acad Sci U S A · 2024
0.80
4
USP11 regulates proliferation and apoptosis of human spermatogonial stem cells via HOXC5-mediated canonical WNT/β-catenin signaling pathway.
PMID: 38722330
Cell Mol Life Sci · 2024
0.70
5
USP11 Exacerbates Radiation-Induced Pneumonitis by Activating Endothelial Cell Inflammatory Response via OTUD5-STING Signaling.
PMID: 38364946
Int J Radiat Oncol Biol Phys · 2024
0.68