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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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USP25
ubiquitin specific peptidase 25
Chromosome 21 · 21q21.1
NCBI Gene: 29761Ensembl: ENSG00000155313.17HGNC: HGNC:12624UniProt: Q9UHP3
111PubMed Papers
21Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
negative regulation of ERAD pathwayprotein deubiquitinationcysteine-type deubiquitinase activityprotein K63-linked deubiquitinationneurodegenerative diseaseinflammatory bowel diseaseCrohn's diseaseulcerative colitis
✦AI Summary

USP25 is a deubiquitinating enzyme that regulates protein stability through removal of ubiquitin chains, with diverse roles in cellular processes and disease pathogenesis. The enzyme exhibits substrate-specific deubiquitinase activity, removing both K48-linked ubiquitin chains (promoting protein stabilization) and K63-linked chains (modulating signaling pathways) 1234. USP25 functions through multiple mechanisms including stabilization of key regulatory proteins: it deubiquitinates SHLD2 to promote DNA repair via non-homologous end joining 2, stabilizes STAT6 to enhance macrophage M2 polarization 3, maintains KRAS protein levels in cancer cells 5, and removes K63-linked ubiquitin from TAB2 to inhibit neuroinflammation 4. Disease relevance includes roles in cancer progression, where USP25 promotes hepatocellular carcinoma through Wnt/β-catenin signaling 6 and maintains oncogenic KRAS signaling 5. Additionally, USP25 contributes to fibrosis development 13, platelet hyperreactivity during aging 7, and provides neuroprotection against ischemic stroke 4. Clinical significance is evidenced by its identification as a potential Parkinson's disease risk locus 8 and its therapeutic potential as a target for cancer treatment and other age-related pathologies.

Sources cited
1
USP25 identified as potential Parkinson's disease risk locus in multi-ancestry GWAS
PMID: 38155330
2
USP25 prevents RAC1 ubiquitination and has anti-inflammatory/anti-fibrotic roles in IgG4-related disease
PMID: 38521787
3
USP25 promotes hepatocellular carcinoma progression via Wnt/β-catenin signaling and interaction with TRIM21
PMID: 37439386
4
USP25 deubiquitinates SHLD2 at K64 to promote NHEJ DNA repair and contributes to chemoresistance
PMID: 38803048
5
USP25 stabilizes STAT6 by reducing K48 ubiquitination, promoting IL-4-induced M2 macrophage polarization and fibrosis
PMID: 39781451
6
USP25 stabilizes talin-1 through deubiquitination, contributing to platelet hyperreactivity during aging
PMID: 40997299
7
USP25 maintains KRAS protein levels by preventing proteasomal degradation in cancer cells
PMID: 40473213
8
USP25 removes K63-linked ubiquitin from TAB2, inhibiting neuroinflammation after cerebral ischemic stroke
PMID: 37587766
Disease Associationsⓘ21
neurodegenerative diseaseOpen Targets
0.32Weak
inflammatory bowel diseaseOpen Targets
0.31Weak
Crohn's diseaseOpen Targets
0.30Weak
ulcerative colitisOpen Targets
0.29Weak
hypothyroidismOpen Targets
0.29Weak
Abruptio PlacentaeOpen Targets
0.29Weak
kidney failureOpen Targets
0.28Weak
autoimmune diseaseOpen Targets
0.25Weak
ovarian neoplasmOpen Targets
0.24Weak
myxedemaOpen Targets
0.24Weak
bladder calculusOpen Targets
0.24Weak
Parkinson diseaseOpen Targets
0.20Weak
Increased total eosinophil countOpen Targets
0.20Weak
frozen shoulderOpen Targets
0.20Weak
ankylosing spondylitisOpen Targets
0.20Weak
sclerosing cholangitisOpen Targets
0.20Weak
asthmaOpen Targets
0.20Weak
smoking initiationOpen Targets
0.20Weak
rheumatoid arthritisOpen Targets
0.19Weak
chronic kidney diseaseOpen Targets
0.19Weak
Epilepsy, idiopathic generalized 19UniProt
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
UBCProtein interaction100%KCTD13Protein interaction98%RIGIProtein interaction94%SUMO2Protein interaction86%SUMO1Protein interaction86%USP13Protein interaction85%
Tissue Expression6 tissues
Heart
100%
Lung
69%
Bone Marrow
55%
Brain
48%
Ovary
44%
Liver
39%
Gene Interaction Network
Click a node to explore
USP25UBCKCTD13RIGISUMO2SUMO1USP13
PROTEIN STRUCTURE
Preparing viewer…
PDB5GP7 · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.64LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.52 [0.42–0.64]
RankingsWhere USP25 stands among ~20K protein-coding genes
  • #4,302of 20,598
    Most Researched111 · top quartile
  • #4,511of 17,882
    Most Constrained (LOEUF)0.64
Genes detectedUSP25
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Multi-ancestry genome-wide association meta-analysis of Parkinson's disease.
PMID: 38155330
Nat Genet · 2024
1.00
2
Expression of USP25 associates with fibrosis, inflammation and metabolism changes in IgG4-related disease.
PMID: 38521787
Nat Commun · 2024
0.90
3
USP25 promotes hepatocellular carcinoma progression by interacting with TRIM21 via the Wnt/β-catenin signaling pathway.
PMID: 37439386
Chin Med J (Engl) · 2023
0.80
4
USP25 Elevates SHLD2-Mediated DNA Double-Strand Break Repair and Regulates Chemoresponse in Cancer.
PMID: 38803048
Adv Sci (Weinh) · 2024
0.70
5
USP25 stabilizes STAT6 to promote IL-4-induced macrophage M2 polarization and fibrosis.
PMID: 39781451
Int J Biol Sci · 2025
0.60