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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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USP13
ubiquitin specific peptidase 13
Chromosome 3 · 3q26.33
NCBI Gene: 8975Ensembl: ENSG00000058056.11HGNC: HGNC:12611UniProt: A0A0A6YZ17
120PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein K29-linked deubiquitinationprotein K6-linked deubiquitinationprotein stabilizationprotein K63-linked deubiquitinationgestational diabetescutaneous lupus erythematosuscolor vision disorderrheumatoid arthritis
✦AI Summary

USP13 is a deubiquitinating enzyme that plays crucial roles in multiple cellular processes including autophagy regulation, cell cycle progression, and tumor development. As a deubiquitinase, USP13 stabilizes key regulatory proteins by removing ubiquitin chains, thereby preventing their proteasomal degradation 12. USP13 promotes autophagy by deubiquitinating BECN1 and stabilizing PIK3C3/VPS34-containing complexes, forming regulatory loops that control autophagy and p53 levels 34. The enzyme demonstrates oncogenic properties across multiple cancer types, including lung squamous cell carcinoma where it drives lineage plasticity in club cells and promotes squamous carcinoma development 5. In renal cell carcinoma, USP13 deubiquitinates and stabilizes ZHX2, contributing to tumorigenesis 1. USP13 also regulates immune responses by deubiquitinating STAT1, enhancing chemokine production and CD8+ T cell infiltration 67. Additionally, USP13 mediates a switch between ferroptosis and autophagy through the NFE2L2/NRF2-SQSTM1/p62-KEAP1 axis in KRAS-mutant lung adenocarcinoma 8. The enzyme's diverse substrate specificity and involvement in cancer progression, autophagy, and immune regulation position USP13 as a potential therapeutic target for various diseases, particularly cancers with dysregulated ubiquitination pathways.

Sources cited
1
USP13 promotes autophagy by stabilizing PIK3C3/VPS34-BECN1 complexes
PMID: 39051473
2
USP13 drives lung squamous cell carcinoma by promoting lineage plasticity in club cells
PMID: 38093367
3
USP13 stabilizes ATG5 through deubiquitination and promotes autophagy in GIST cells
PMID: 36528756
4
USP13 deubiquitinates and stabilizes STAT1 in cardiomyocytes, protecting against hypertrophy
PMID: 40593642
5
USP13 facilitates ferroptosis-to-autophagy switch through NFE2L2/NRF2-SQSTM1/p62-KEAP1 axis
PMID: 39360581
6
USP13 competes with MBTPS1 for STAT1 binding, enhancing antitumor immunity
PMID: 40307212
7
USP13 deubiquitinates WWP1 and is transcriptionally regulated by YY1 in hepatocellular carcinoma
PMID: 40319251
8
USP13 promotes deubiquitination of ZHX2 and contributes to kidney cancer tumorigenesis
PMID: 36037364
Disease Associationsⓘ20
gestational diabetesOpen Targets
0.31Weak
cutaneous lupus erythematosusOpen Targets
0.27Weak
color vision disorderOpen Targets
0.24Weak
rheumatoid arthritisOpen Targets
0.22Weak
Abnormality of the gastrointestinal tractOpen Targets
0.22Weak
neoplasmOpen Targets
0.12Weak
hepatocellular carcinomaOpen Targets
0.11Weak
colorectal carcinomaOpen Targets
0.10Weak
ovarian cancerOpen Targets
0.10Weak
breast cancerOpen Targets
0.09Suggestive
cancerOpen Targets
0.09Suggestive
cardiac hypertrophyOpen Targets
0.09Suggestive
diffuse large B-cell lymphomaOpen Targets
0.09Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.08Suggestive
cervical cancerOpen Targets
0.08Suggestive
posterior cortical atrophyOpen Targets
0.08Suggestive
osteosarcomaOpen Targets
0.08Suggestive
idiopathic pulmonary fibrosisOpen Targets
0.08Suggestive
oral squamous cell carcinomaOpen Targets
0.08Suggestive
small cell lung carcinomaOpen Targets
0.08Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NPLOC4Protein interaction100%UBCProtein interaction99%YOD1Protein interaction99%SIAH2Protein interaction95%ATXN3Protein interaction95%PTENProtein interaction91%
Tissue Expression6 tissues
Heart
100%
Brain
17%
Ovary
12%
Lung
10%
Liver
6%
Bone Marrow
1%
Gene Interaction Network
Click a node to explore
USP13NPLOC4UBCYOD1SIAH2ATXN3PTEN
PROTEIN STRUCTURE
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PDB2L80 · NMR
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.77LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.63 [0.51–0.77]
RankingsWhere USP13 stands among ~20K protein-coding genes
  • #3,947of 20,598
    Most Researched120 · top quartile
  • #6,195of 17,882
    Most Constrained (LOEUF)0.77
Genes detectedUSP13
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Spautin-1 promotes PINK1-PRKN-dependent mitophagy and improves associative learning capability in an alzheimer disease animal model.
PMID: 39051473
Autophagy · 2024
1.00
2
USP13 drives lung squamous cell carcinoma by switching lung club cell lineage plasticity.
PMID: 38093367
Mol Cancer · 2023
0.90
3
N
PMID: 36528756
Cell Death Differ · 2023
0.80
4
Cardiomyocyte-derived USP13 protects hearts from hypertrophy via deubiquitinating and stabilizing STAT1 in male mice.
PMID: 40593642
Nat Commun · 2025
0.70
5
USP13 facilitates a ferroptosis-to-autophagy switch by activation of the NFE2L2/NRF2-SQSTM1/p62-KEAP1 axis dependent on the KRAS signaling pathway.
PMID: 39360581
Autophagy · 2025
0.60