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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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UCHL3
ubiquitin C-terminal hydrolase L3
Chromosome 13 · 13q22.2
NCBI Gene: 7347Ensembl: ENSG00000118939.20HGNC: HGNC:12515UniProt: A0A140VJZ4
121PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein deubiquitinationpeptidase activitypositive regulation of double-strand break repair via homologous recombinationprotein bindingAbnormality of the skeletal systemliver diseasenon-small cell lung carcinomahepatocellular carcinoma
✦AI Summary

UCHL3 (ubiquitin C-terminal hydrolase L3) is a deubiquitinating enzyme (DUB) that processes ubiquitin precursors and removes ubiquitin modifications from target proteins 1. As a cysteine protease, UCHL3 exhibits catalytic preferences for Lys-48-linked ubiquitin chains and possesses both deubiquitination and deneddylation activities 1. UCHL3 plays critical roles in fundamental cellular processes including cell cycle regulation, DNA repair, and protein homeostasis 1. Mechanistically, UCHL3 stabilizes diverse oncoproteins through deubiquitination-dependent interactions. In non-small cell lung cancer, UCHL3 deubiquitylates and stabilizes the aryl hydrocarbon receptor (AhR), promoting stemness genes including ABCG2, KLF4, and c-Myc 2. Similarly, in ovarian cancer, UCHL3 removes K48-linked ubiquitin chains from RIPK4 at the K469 site, enhancing its stabilization and promoting metastasis 3. In anaplastic thyroid cancer, UCHL3 stabilizes YAP through deubiquitylation, activating Hippo pathway signaling 4. In hepatocellular carcinoma, UCHL3 stabilizes both EEF1A1 and PKM2 through deubiquitination 56. UCHL3 demonstrates paradoxical roles in disease: while functioning as an oncogene in multiple cancer types 7, it is required for normal stress response maintenance in retinal, skeletal muscle, and germ cells 1. Dysregulation associates with neurodegenerative diseases, learning deficits, and cellular stress responses 1. UCHL3 inhibitors show therapeutic promise for cancer treatment by reducing oncoprotein stability 23.

Sources cited
1
UCHL3 is a cysteine protease with deubiquitination and deneddylation activities involved in cell cycle regulation, DNA repair, apoptosis, and associated with neurodegenerative diseases and learning deficits
PMID: 39226739
2
UCHL3 deubiquitylates and stabilizes AhR in lung cancer, promoting stemness genes ABCG2, KLF4, and c-Myc
PMID: 32546741
3
UCHL3 deubiquitinates RIPK4 at K469 site, removing K48-linked ubiquitin chains and enhancing RIPK4 stabilization in ovarian cancer metastasis
PMID: 38664501
4
UCHL3 stabilizes YAP through deubiquitylation, promoting anaplastic thyroid cancer progression via Hippo pathway signaling
PMID: 36813921
5
UCHL3 stabilizes EEF1A1 through deubiquitination, promoting hepatocellular carcinoma migration, stemness, and drug resistance
PMID: 38965582
6
UCHL3 deubiquitinates and stabilizes PKM2 at K206 site, enhancing cuproptosis and pyruvate biosynthesis in hepatocellular carcinoma
PMID: 40451468
7
UCHL3 exhibits paradoxical roles as both tumor suppressor and oncogene across different cancer types and enhances DNA damage repair-mediated chemotherapy/radiotherapy resistance
PMID: 40691601
Disease Associationsⓘ20
Abnormality of the skeletal systemOpen Targets
0.41Moderate
liver diseaseOpen Targets
0.31Weak
non-small cell lung carcinomaOpen Targets
0.10Suggestive
hepatocellular carcinomaOpen Targets
0.10Suggestive
neoplasmOpen Targets
0.09Suggestive
colorectal carcinomaOpen Targets
0.08Suggestive
gastric cancerOpen Targets
0.08Suggestive
urinary bladder carcinomaOpen Targets
0.08Suggestive
X-linked retinal dysplasiaOpen Targets
0.08Suggestive
age-related macular degenerationOpen Targets
0.07Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.07Suggestive
retinitis pigmentosaOpen Targets
0.06Suggestive
choroidal dystrophy, central areolar, 1Open Targets
0.06Suggestive
lung cancerOpen Targets
0.06Suggestive
Familial drusenOpen Targets
0.06Suggestive
melanomaOpen Targets
0.05Suggestive
severe early-childhood-onset retinal dystrophyOpen Targets
0.05Suggestive
Stargardt diseaseOpen Targets
0.05Suggestive
MODYOpen Targets
0.05Suggestive
ThrombocytopeniaOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
PSMA2Protein interaction100%USP14Protein interaction100%PSMD14Protein interaction100%NAE1Protein interaction100%UBA3Protein interaction100%NEDD8Protein interaction97%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
92%
Brain
62%
Heart
50%
Lung
38%
Ovary
20%
Gene Interaction Network
Click a node to explore
UCHL3PSMA2USP14PSMD14NAE1UBA3NEDD8
PROTEIN STRUCTURE
Preparing viewer…
PDB1XD3 · 1.45 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.94LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.61 [0.41–0.94]
RankingsWhere UCHL3 stands among ~20K protein-coding genes
  • #3,903of 20,598
    Most Researched121 · top quartile
  • #8,782of 17,882
    Most Constrained (LOEUF)0.94
Genes detectedUCHL3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
MLKL-USP7-UBA52 signaling is indispensable for autophagy in brain through maintaining ubiquitin homeostasis.
PMID: 39193909
Autophagy · 2025
1.00
2
Role of UCHL3 in health and disease.
PMID: 39226739
Biochem Biophys Res Commun · 2024
0.90
3
The deubiquitylase UCHL3 maintains cancer stem-like properties by stabilizing the aryl hydrocarbon receptor.
PMID: 32546741
Signal Transduct Target Ther · 2020
0.80
4
GSK3β and UCHL3 govern RIPK4 homeostasis via deubiquitination to enhance tumor metastasis in ovarian cancer.
PMID: 38664501
Oncogene · 2024
0.70
5
The deubiquitinating enzyme UCHL3 promotes anaplastic thyroid cancer progression and metastasis through Hippo signaling pathway.
PMID: 36813921
Cell Death Differ · 2023
0.60