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26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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USP14
ubiquitin specific peptidase 14
Chromosome 18 · 18p11.32
NCBI Gene: 9097Ensembl: ENSG00000101557.16HGNC: HGNC:12612UniProt: P54578
229PubMed Papers
20Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneProtease
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolcysteine-type deubiquitinase activityendopeptidase inhibitor activityprotein bindingAbnormality of the skeletal systemdistal arthrogryposisdiabetes mellituscomplex neurodevelopmental disorder
✦AI Summary

USP14 is a proteasome-associated deubiquitinase that removes ubiquitin from proteasome-targeted proteins, ensuring ubiquitin regeneration at the proteasome 1. As a reversibly associated proteasomal subunit, USP14 exists in both proteasome-bound and free cellular forms 2. Beyond proteasomal protein degradation, USP14 plays diverse regulatory roles: it is required for CXCR4 chemokine receptor degradation critical for cell chemotaxis 3, serves as a physiological inhibitor of ER-associated degradation 4, and stabilizes the viral DNA sensor cGAS to enhance innate immune defense 5. USP14 inhibits OPTN-mediated autophagic degradation of KDM4D, thereby negatively regulating histone methylation marks 1. Clinically, USP14 dysregulation contributes to multiple diseases. In hepatocellular carcinoma, USP14-mediated TUBA1A K370 deubiquitination promotes tumorigenesis through AKT activation, with targeting this axis blocking liver cancer in vivo 6. USP14 overexpression exacerbates nonalcoholic fatty liver disease progression via HSP90AA1-stabilization-dependent CYP2E1 accumulation 7. In atherosclerosis, USP14-mediated cGAS stabilization promotes pro-inflammatory macrophage polarization 8. Conversely, USP14 inhibition promotes DNA damage repair and suppresses granulosa cell senescence in premature ovarian insufficiency 9. These findings position USP14 as a promising therapeutic target across multiple pathologies.

Sources cited
1
USP14 removes ubiquitin from proteasome-targeted proteins and inhibits OPTN-mediated autophagic degradation of KDM4D
PMID: 35145029
2
USP14 ensures ubiquitin regeneration at the proteasome and exists as reversibly associated proteasomal subunit with proteasome-free cellular pools
PMID: 18162577
3
USP14 is required for CXCR4 degradation critical for CXCL12-induced cell chemotaxis
PMID: 19106094
4
USP14 serves as physiological inhibitor of ER-associated degradation by inhibiting unfolded protein degradation via ERN1 interaction
PMID: 19135427
5
USP14 plays role in innate immune defense against viruses by stabilizing cGAS and inhibiting its autophagic degradation
PMID: 27666593
6
USP14-mediated TUBA1A K370 deubiquitination drives hepatocellular carcinoma through AKT activation; targeting AKT-USP14-TUBA1A complex blocks liver tumorigenesis
PMID: 39348425
7
USP14 overexpression exacerbates nonalcoholic fatty liver disease by stabilizing HSP90AA1 and promoting CYP2E1 accumulation and hepatic oxidative stress
PMID: 37633951
8
USP14 promotes cGAS K48-linked polyubiquitination degradation; USP14 reduction alleviates pro-inflammatory macrophage responses and protects against atherosclerosis
PMID: 39178733
9
USP14 inhibition promotes DNA damage repair function and suppresses granulosa cell senescence in premature ovarian insufficiency models
PMID: 39261935
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.39Weak
distal arthrogryposisOpen Targets
0.37Weak
diabetes mellitusOpen Targets
0.27Weak
complex neurodevelopmental disorderOpen Targets
0.18Weak
neoplasmOpen Targets
0.11Weak
gastric cancerOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
breast cancerOpen Targets
0.10Weak
head and neck squamous cell carcinomaOpen Targets
0.10Weak
esophageal squamous cell carcinomaOpen Targets
0.10Weak
colorectal carcinomaOpen Targets
0.10Weak
cancerOpen Targets
0.10Weak
non-small cell lung carcinomaOpen Targets
0.10Suggestive
glioblastoma multiformeOpen Targets
0.09Suggestive
oral squamous cell carcinomaOpen Targets
0.09Suggestive
lung cancerOpen Targets
0.09Suggestive
colorectal cancerOpen Targets
0.08Suggestive
prostate cancerOpen Targets
0.08Suggestive
Parkinson diseaseOpen Targets
0.08Suggestive
endometrial cancerOpen Targets
0.08Suggestive
Pathogenic Variants1
NM_005151.4(USP14):c.233_236del (p.Leu78fs)Pathogenic
See cases|Distal arthrogryposis and CNS involvement|not provided
★☆☆☆2022→ Residue 78
View on ClinVar ↗
Related Genes
PSMD10Protein interaction100%RAD23AProtein interaction100%UCHL3Protein interaction100%SEM1Protein interaction100%PSMD14Protein interaction100%PSMB9Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
68%
Heart
57%
Lung
41%
Liver
41%
Ovary
38%
Gene Interaction Network
Click a node to explore
USP14PSMD10RAD23AUCHL3SEM1PSMD14PSMB9
PROTEIN STRUCTURE
Preparing viewer…
PDB9F6G · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.63LoF Tolerant
pLIⓘ
0.08Tolerant
Observed/Expected LoF0.44 [0.31–0.63]
RankingsWhere USP14 stands among ~20K protein-coding genes
  • #1,765of 20,598
    Most Researched229 · top 10%
  • #4,709of 5,498
    Most Pathogenic Variants1
  • #4,415of 17,882
    Most Constrained (LOEUF)0.63 · top quartile
Genes detectedUSP14
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
Copper metabolism in cell death and autophagy.
PMID: 37055935
Autophagy · 2023
1.00
2
Integrated ubiquitomics characterization of hepatocellular carcinomas.
PMID: 39348425
Hepatology · 2025
0.90
3
Targeting cancer-associated fibroblast autophagy renders pancreatic cancer eradicable with immunochemotherapy by inhibiting adaptive immune resistance.
PMID: 38174993
Autophagy · 2024
0.80
4
USP14 governs CYP2E1 to promote nonalcoholic fatty liver disease through deubiquitination and stabilization of HSP90AA1.
PMID: 37633951
Cell Death Dis · 2023
0.70
5
Deubiquitination of CIB1 by USP14 promotes lenvatinib resistance via the PAK1-ERK1/2 axis in hepatocellular carcinoma.
PMID: 38993552
Int J Biol Sci · 2024
0.64