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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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USP50
ubiquitin specific peptidase 50
Chromosome 15 · 15q21.2
NCBI Gene: 373509Ensembl: ENSG00000170236.16HGNC: HGNC:20079UniProt: Q70EL3
21PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
nuclear speck organizationpositive regulation of NLRP3 inflammasome complex assemblycysteine-type deubiquitinase activitynucleusosteoarthritis, kneeosteoarthritishypertensionosteoarthritis, hip
✦AI Summary

USP50 is a deubiquitinating enzyme that removes conjugated ubiquitin from specific protein substrates to regulate multiple cellular processes. Mechanistically, USP50 cleaves lysine-63-linked polyubiquitin chains on target proteins, with well-characterized substrates including the PYCARD/ASC inflammasome adapter protein 1, the ACE2 receptor 2, and carnitine palmitoyltransferase 1a (CPT1a) 3. USP50 promotes inflammasome activation by deubiquitinating ASC, enhancing NLRP3-mediated IL-1β and IL-18 secretion 1. However, USP50 also suppresses NLRP3 inflammasome-driven pyroptosis in sepsis-induced acute lung injury through K48-linked ubiquitination-mediated NLRP3 degradation 4. Beyond inflammation, USP50 stabilizes CPT1a to drive fatty acid oxidation in LPS-treated macrophages 3 and regulates replication fork dynamics by promoting WRN-FEN1 localization while suppressing DNA2 nuclease and RECQL helicase activities 5. Clinically, USP50 upregulation associates with sepsis-induced acute respiratory distress syndrome 4, duodenogastric reflux-induced gastric tumorigenesis 6, and tendinopathy pathogenesis 7. USP50 represents a potential therapeutic target for inflammatory and infectious diseases.

Sources cited
1
USP50 deubiquitinates K63-linked polyubiquitination of ASC to regulate NLRP3 inflammasome activation and IL-1β/IL-18 secretion
PMID: 28094437
2
USP50 is a deubiquitinase that decreases ACE2 ubiquitination and regulates ACE2 protein levels
PMID: 37608279
3
USP50 interacts with and stabilizes CPT1a by preventing its ubiquitination and degradation, promoting fatty acid oxidation in macrophages
PMID: 34976224
4
USP50 upregulation occurs in sepsis-induced acute respiratory distress syndrome and USP50 suppression reduces NLRP3-driven macrophage pyroptosis
PMID: 40702108
5
USP50 is a chromatin-associated protein required for DNA replication fork progression and telomere maintenance by promoting WRN-FEN1 localization
PMID: 39284827
6
USP50 deubiquitinates ASC to activate NLRP3 inflammasome in duodenogastric reflux-induced gastric carcinogenesis
PMID: 38469295
7
USP50 cleaves K63-linked ubiquitin chains on PYCARD/ASC, blocking selective autophagic degradation of the AIM2 inflammasome complex in tendinopathy
PMID: 37647255
8
USP50 levels increase during erythropoiesis and associate with regulation of Ku70 protein stability
PMID: 29101126
Disease Associationsⓘ20
osteoarthritis, kneeOpen Targets
0.14Weak
osteoarthritisOpen Targets
0.14Weak
hypertensionOpen Targets
0.12Weak
osteoarthritis, hipOpen Targets
0.11Weak
joint diseaseOpen Targets
0.10Weak
hepatocellular carcinomaOpen Targets
0.07Suggestive
total knee arthroplastyOpen Targets
0.07Suggestive
Familial ocular anterior segment mesenchymal dysgenesisOpen Targets
0.06Suggestive
Persistent pupillary membraneOpen Targets
0.05Suggestive
Axenfeld anomalyOpen Targets
0.04Suggestive
Rieger anomalyOpen Targets
0.04Suggestive
iridocorneal endothelial syndromeOpen Targets
0.04Suggestive
SepsisOpen Targets
0.04Suggestive
posterior amorphous corneal dystrophyOpen Targets
0.04Suggestive
isolated ectopia lentisOpen Targets
0.04Suggestive
exfoliation syndromeOpen Targets
0.04Suggestive
X-linked corneal dermoidOpen Targets
0.04Suggestive
posterior polymorphous corneal dystrophyOpen Targets
0.04Suggestive
X-linked intellectual disability-retinitis pigmentosa syndromeOpen Targets
0.03Suggestive
posterior polymorphous corneal dystrophy 1Open Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HSP90AB1Protein interaction100%PAN2Protein interaction73%USP47Protein interaction70%ZNF354AProtein interaction70%STMP1Shared pathway22%USP8Shared pathway19%
Tissue Expression6 tissues
Liver
100%
Bone Marrow
70%
Brain
30%
Lung
26%
Ovary
26%
Heart
0%
Gene Interaction Network
Click a node to explore
USP50HSP90AB1PAN2USP47ZNF354ASTMP1USP8
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q70EL3
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
1.27LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.97 [0.75–1.27]
RankingsWhere USP50 stands among ~20K protein-coding genes
  • #14,062of 20,598
    Most Researched21
  • #13,385of 17,882
    Most Constrained (LOEUF)1.27
Genes detectedUSP50
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Mitochondrial STAT3 exacerbates LPS-induced sepsis by driving CPT1a-mediated fatty acid oxidation.
PMID: 34976224
Theranostics · 2022
1.00
2
Pristimerin suppresses AIM2 inflammasome by modulating AIM2-PYCARD/ASC stability via selective autophagy to alleviate tendinopathy.
PMID: 37647255
Autophagy · 2024
0.90
3
USP50 suppresses alternative RecQ helicase use and deleterious DNA2 activity during replication.
PMID: 39284827
Nat Commun · 2024
0.80
4
USP50 regulates NLRP3 inflammasome activation in duodenogastric reflux-induced gastric tumorigenesis.
PMID: 38469295
Front Immunol · 2024
0.70
5
ACE2 in chronic disease and COVID-19: gene regulation and post-translational modification.
PMID: 37608279
J Biomed Sci · 2023
0.60