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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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CASP4
caspase 4
Chromosome 11 · 11q22.3
NCBI Gene: 837Ensembl: ENSG00000196954.14HGNC: HGNC:1505UniProt: P49662
124PubMed Papers
20Diseases
1Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
ApoptosisProtease
CLINICAL
Clinical Trials
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cysteine-type endopeptidase activitycytosolmitochondrionprotein bindingbacterial diseaseAlzheimer diseaseneoplasmcirrhosis of liver
✦AI Summary

CASP4 (caspase-4) is an inflammatory caspase that serves as a cytosolic pattern recognition receptor for lipopolysaccharide (LPS) from Gram-negative bacteria 1. The protein directly binds LPS through its CARD domain with high specificity and affinity, inducing oligomerization and autoprocessing to generate an active p10 form 12. Once activated, CASP4 cleaves gasdermin D (GSDMD) to trigger pyroptosis, a form of inflammatory cell death characterized by membrane pore formation and cytokine release 34. CASP4 can also directly process pro-IL-18 with efficiency similar to caspase-1, enabling IL-18 release independently of canonical inflammasomes 5. The enzyme plays critical roles in host defense against bacterial infections and inflammatory diseases. In blood-brain barrier endothelial cells, CASP4 activation leads to barrier breakdown during sepsis 6. However, some pathogens like Shigella flexneri evade CASP4-mediated immunity through ADP-riboxanation modifications that block caspase autoprocessing and GSDMD cleavage 7. Therapeutically, CASP4-mediated pyroptosis can be inhibited by disulfiram, which blocks GSDMD pore formation 4.

Sources cited
1
CASP4 directly binds LPS through CARD domain and undergoes oligomerization upon binding
PMID: 25119034
2
Autoprocessing generates p10 form required for GSDMD cleavage and pyroptosis
PMID: 32109412
3
CASP4/caspase-11 cleaves gasdermin D for pyroptosis and IL-1β maturation
PMID: 26375259
4
Disulfiram inhibits pyroptosis by blocking GSDMD pore formation
PMID: 32367036
5
CASP4 activation in brain endothelial cells mediates blood-brain barrier breakdown
PMID: 38632402
6
CASP4 can directly cleave pro-IL-18 independently of canonical inflammasomes
PMID: 37993712
7
Shigella evades CASP4 through ADP-riboxanation that blocks autoprocessing
PMID: 34671164
Disease Associationsⓘ20
bacterial diseaseOpen Targets
0.51Moderate
Alzheimer diseaseOpen Targets
0.11Weak
neoplasmOpen Targets
0.11Weak
cirrhosis of liverOpen Targets
0.11Weak
non-small cell lung carcinomaOpen Targets
0.11Weak
risk-taking behaviourOpen Targets
0.10Suggestive
gliomaOpen Targets
0.10Suggestive
Hepatic fibrosisOpen Targets
0.10Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.09Suggestive
infectionOpen Targets
0.08Suggestive
lung cancerOpen Targets
0.08Suggestive
colorectal carcinomaOpen Targets
0.08Suggestive
chronic hepatitis C virus infectionOpen Targets
0.07Suggestive
diabetes mellitusOpen Targets
0.07Suggestive
periodontitisOpen Targets
0.06Suggestive
chronic kidney diseaseOpen Targets
0.06Suggestive
achalasia-alacrima syndromeOpen Targets
0.06Suggestive
hyperinsulinism due to INSR deficiencyOpen Targets
0.05Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.05Suggestive
MODYOpen Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Drug Targets1
EMRICASANPhase II
Caspase inhibitor
Hepatic fibrosis
Related Genes
PYCARDProtein interaction100%AIM2Protein interaction100%NLRC4Protein interaction100%IL1BProtein interaction94%IL18Protein interaction93%SCAF11Protein interaction86%
Tissue Expression6 tissues
Lung
100%
Liver
52%
Ovary
34%
Bone Marrow
23%
Heart
14%
Brain
5%
Gene Interaction Network
Click a node to explore
CASP4PYCARDAIM2NLRC4IL1BIL18SCAF11
PROTEIN STRUCTURE
Preparing viewer…
PDB7WR6 · 1.96 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.98LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.65 [0.44–0.98]
RankingsWhere CASP4 stands among ~20K protein-coding genes
  • #3,786of 20,598
    Most Researched124 · top quartile
  • #9,358of 17,882
    Most Constrained (LOEUF)0.98
Genes detectedCASP4
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
FDA-approved disulfiram inhibits pyroptosis by blocking gasdermin D pore formation.
PMID: 32367036
Nat Immunol · 2020
1.00
2
Brain endothelial GSDMD activation mediates inflammatory BBB breakdown.
PMID: 38632402
Nature · 2024
0.90
3
Inflammatory caspases are innate immune receptors for intracellular LPS.
PMID: 25119034
Nature · 2014
0.80
4
The AIM2 inflammasome exacerbates atherosclerosis in clonal haematopoiesis.
PMID: 33731931
Nature · 2021
0.70
5
Caspase-11 cleaves gasdermin D for non-canonical inflammasome signalling.
PMID: 26375259
Nature · 2015
0.60