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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZUP1
zinc finger containing ubiquitin peptidase 1
Chromosome 6 · 6q22.1
NCBI Gene: 221302Ensembl: ENSG00000153975.11HGNC: HGNC:21224UniProt: A0A0S2Z644
24PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Hub GeneTranscription Factor
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolprotein bindingnucleoplasmnucleusovarian dysfunctiontransient ischemic attackcancercolorectal carcinoma
✦AI Summary

ZUP1 (zinc finger containing ubiquitin peptidase 1) is a deubiquitinating enzyme (DUB) that defines a previously unrecognized class of evolutionarily conserved cysteine protease DUBs 1. The protein exhibits high selectivity for cleaving long K63-linked polyubiquitin chains through its tandem ubiquitin-binding domains, while showing poor activity against mono- or di-ubiquitin substrates and weak activity against K11- and K48-linked chains 231. ZUP1 has a modular architecture with four N-terminal zinc finger domains and a C-terminal deubiquitinase domain that shares structural similarity with UFM1- and Atg8-specific proteases 24. The enzyme plays crucial roles in genome stability by being recruited to genotoxic stress sites and regulating K63-ubiquitin conjugates during replication stress, promoting chromosome 6 in a catalytically-dependent manner 1. Additionally, ZUP1 modulates ubiquitination of replication protein A (RPA) complex proteins in response to replication stress 3. Recent studies have revealed ZUP1's function in innate immunity as a key component of the MAVS complex, where it enhances antiviral signaling by binding viral RNA through its zinc finger domains and facilitating type I interferon production 45. ZUP1 expression is upregulated during viral infection, and its deficiency increases susceptibility to viral infections 4.

Sources cited
1
ZUP1 defines a new class of DUBs, selectively cleaves K63-linked chains, and promotes genome stability
PMID: 29576528
2
ZUP1 shares structural fold with UFM1/Atg8 proteases and uses modular UBDs for K63 specificity
PMID: 29476094
3
ZUP1 shows high selectivity for K63-linked chains and modulates RPA ubiquitination
PMID: 29563501
4
ZUP1 is a MAVS complex component with zinc finger domains that bind viral RNA and enhance antiviral immunity
PMID: 40095368
5
ZUP1 acts as positive regulator of RLR signaling and facilitates ISG transcription through chromatin remodeling
PMID: 38460305
Disease Associationsⓘ20
ovarian dysfunctionOpen Targets
0.34Weak
transient ischemic attackOpen Targets
0.21Weak
cancerOpen Targets
0.08Suggestive
colorectal carcinomaOpen Targets
0.08Suggestive
colitisOpen Targets
0.07Suggestive
hepatocellular carcinomaOpen Targets
0.06Suggestive
posterior cortical atrophyOpen Targets
0.05Suggestive
head and neck squamous cell carcinomaOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.05Suggestive
triple-negative breast cancerOpen Targets
0.04Suggestive
non-small cell lung carcinomaOpen Targets
0.04Suggestive
infectionOpen Targets
0.04Suggestive
breast cancerOpen Targets
0.04Suggestive
cervical cancerOpen Targets
0.03Suggestive
glioblastoma multiformeOpen Targets
0.03Suggestive
gliomaOpen Targets
0.03Suggestive
gastric cancerOpen Targets
0.03Suggestive
melanomaOpen Targets
0.03Suggestive
atrial fibrillationOpen Targets
0.03Suggestive
atrial flutterOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
RPS27AProtein interaction95%OTUD7BProtein interaction95%STAMBPL1Protein interaction95%WDR48Protein interaction95%OTULINProtein interaction95%JOSD2Protein interaction95%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
34%
Lung
31%
Liver
23%
Heart
16%
Ovary
14%
Gene Interaction Network
Click a node to explore
ZUP1RPS27AOTUD7BSTAMBPL1WDR48OTULINJOSD2
PROTEIN STRUCTURE
Preparing viewer…
PDB6EI1 · 1.73 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.12LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.86 [0.68–1.12]
RankingsWhere ZUP1 stands among ~20K protein-coding genes
  • #13,363of 20,598
    Most Researched24
  • #11,530of 17,882
    Most Constrained (LOEUF)1.12
Genes detectedZUP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A structural basis for the diverse linkage specificities within the ZUFSP deubiquitinase family.
PMID: 35058438
Nat Commun · 2022
1.00
2
ZUP1 is a key component of the MAVS complex and acts as a protector of host against viral invasion.
PMID: 40095368
FASEB J · 2025
0.90
3
Reactive-site-centric chemoproteomics identifies a distinct class of deubiquitinase enzymes.
PMID: 29563501
Nat Commun · 2018
0.80
4
MAVS promotes interferon signaling in RNA virus infection by ZUFSP-mediated chromatin regulation.
PMID: 38460305
Int Immunopharmacol · 2024
0.70
5
Friend or Foe: UCHL3 Mediated Carcinogenesis and Current Approaches in Small Molecule Inhibitors' Development.
PMID: 34238144
Curr Med Chem · 2021
0.60