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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ZBP1
Z-DNA binding protein 1
Chromosome 20 · 20q13.31
NCBI Gene: 81030Ensembl: ENSG00000124256.16HGNC: HGNC:16176UniProt: Q9H171
74PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
RNA bindingDNA bindingdefense response to viruscytoplasmneoplasminfectionsystemic lupus erythematosusSepsis
✦AI Summary

Z-DNA binding protein 1 (ZBP1) is a cytosolic nucleic acid sensor that functions as a master regulator of innate immune responses and inflammatory cell death. ZBP1 recognizes pathogen-associated molecular patterns including bacterial DNA, viral Z-RNA, and Z-DNA through its Zα-containing domains 12. Upon activation, ZBP1 initiates type I interferon production and triggers RIPK3-mediated necroptosis and PANoptosis (a coordinated inflammatory cell death involving apoptosis, necroptosis, and pyroptosis) 123. ZBP1 operates within a multi-protein PANoptosome complex containing AIM2, pyrin, caspase-1, caspase-8, RIPK3, and FADD, providing coordinated host defense against infections including herpes simplex virus 1 and Francisella novicida 3. During influenza infection, ZBP1 senses viral Z-RNA in the nucleus, triggering nuclear MLKL activation that disrupts the nuclear envelope and activates neutrophils 2. ADAR1 negatively regulates ZBP1-mediated PANoptosis through Zα domain interactions, and this suppression promotes tumorigenesis 4. Beyond pathogenic sensing, ZBP1 mediates heat stress-induced cell death independent of nucleic acid sensing 5. These functions establish ZBP1 as a critical sentinel balancing host defense against infection with potential pathological consequences in severe disease and cancer development.

Sources cited
1
ZBP1 functions as a DNA sensor inducing type I interferon production and partially contributes to IFN-β expression upon bacterial infection
PMID: 39850894
2
ZBP1 senses influenza A virus Z-RNA and initiates RIPK3-mediated MLKL activation leading to necroptosis with nuclear envelope disruption
PMID: 32200799
3
ZBP1 forms part of the AIM2 PANoptosome complex with multiple inflammasome sensors to drive PANoptosis during viral infections
PMID: 34471287
4
ADAR1 suppresses ZBP1-mediated PANoptosis through interactions with ZBP1's Zα2 domain, promoting tumorigenesis
PMID: 34686350
5
ZBP1 mediates heat stress-induced cell death through RIPK3-dependent mechanisms independent of nucleic acid sensing
PMID: 35511979
Disease Associationsⓘ20
neoplasmOpen Targets
0.11Weak
infectionOpen Targets
0.11Weak
systemic lupus erythematosusOpen Targets
0.10Suggestive
SepsisOpen Targets
0.10Suggestive
Herpes ZosterOpen Targets
0.08Suggestive
femoral neck fractureOpen Targets
0.08Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.08Suggestive
papillary thyroid carcinomaOpen Targets
0.08Suggestive
oral squamous cell carcinomaOpen Targets
0.07Suggestive
fungal infectious diseaseOpen Targets
0.07Suggestive
COVID-19Open Targets
0.06Suggestive
breast angiosarcomaOpen Targets
0.06Suggestive
acute lung injuryOpen Targets
0.05Suggestive
periodontitisOpen Targets
0.05Suggestive
head and neck squamous cell carcinomaOpen Targets
0.05Suggestive
Inflammatory abnormality of the skinOpen Targets
0.05Suggestive
myocardial infarctionOpen Targets
0.05Suggestive
cancerOpen Targets
0.05Suggestive
age-related macular degenerationOpen Targets
0.04Suggestive
viral diseaseOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
MLKLProtein interaction100%CASP8Protein interaction100%IKBKEProtein interaction100%TBK1Protein interaction100%RIPK1Protein interaction100%IRF3Protein interaction99%
Tissue Expression6 tissues
Bone Marrow
100%
Lung
23%
Liver
5%
Heart
2%
Brain
1%
Ovary
1%
Gene Interaction Network
Click a node to explore
ZBP1MLKLCASP8IKBKETBK1RIPK1IRF3
PROTEIN STRUCTURE
Preparing viewer…
PDB3EYI · 1.45 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.29LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.00 [0.79–1.29]
RankingsWhere ZBP1 stands among ~20K protein-coding genes
  • #6,440of 20,598
    Most Researched74
  • #13,625of 17,882
    Most Constrained (LOEUF)1.29
Genes detectedZBP1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
ZBP1 senses
PMID: 39850894
Front Immunol · 2024
1.00
2
Influenza Virus Z-RNAs Induce ZBP1-Mediated Necroptosis.
PMID: 32200799
Cell · 2020
0.90
3
AIM2 forms a complex with pyrin and ZBP1 to drive PANoptosis and host defence.
PMID: 34471287
Nature · 2021
0.80
4
ADAR1 restricts ZBP1-mediated immune response and PANoptosis to promote tumorigenesis.
PMID: 34686350
Cell Rep · 2021
0.70
5
ADAR1 and ZBP1 in innate immunity, cell death, and disease.
PMID: 36710220
Trends Immunol · 2023
0.60