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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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PELI2
pellino E3 ubiquitin protein ligase family member 2
Chromosome 14 · 14q22.3
NCBI Gene: 57161Ensembl: ENSG00000139946.11HGNC: HGNC:8828UniProt: Q9H716
34PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
positive regulation of protein phosphorylationpositive regulation of MAPK cascadeprotein bindingprotein polyubiquitinationalcohol drinkingmathematical abilitysmoking initiationulcerative colitis
✦AI Summary

PELI2 is an E3 ubiquitin ligase that functions as a critical regulator of innate immunity and cellular differentiation through lysine-63-linked polyubiquitination of target proteins 1. Mechanistically, PELI2 inhibits STING signaling by binding phosphorylated STING and ubiquitinating TBK1, thereby setting a threshold for interferon production that protects against aberrant immune activation 2. During viral infection, STING-induced downregulation of PELI2 creates a positive feedback loop enabling robust antiviral responses. In lymphopoiesis, PELI2 stabilizes PU.1 and TCF3 via K63-polyubiquitination to regulate IL-7R expression, which is essential for B-cell development 1. Dysregulated PELI2 contributes to disease pathogenesis: insufficient PELI2 in lupus patients correlates with elevated interferon production 2, while a Thr257Ile variant causes constitutive NLRP3 inflammasome activation in ocular pterygium-digital keloid dysplasia 3. PELI2 loss is associated with poor prognosis in colorectal cancer, where PELI2 suppresses tumor progression via MAPK pathway inhibition 4. Additionally, PELI2 downregulation promotes B-cell acute lymphoblastic leukemia proliferation 1, and elevated PELI2 contributes to acute lung injury through inflammatory amplification 5. These findings indicate PELI2 functions as both an immune checkpoint and tumor suppressor with significant therapeutic potential.

Sources cited
1
PELI2 inhibits STING signaling and IRF3 by ubiquitinating TBK1; dysregulation contributes to lupus and interferonopathies
PMID: 38917796
2
PELI2 stabilizes PU.1 and TCF3 via K63-polyubiquitination to regulate IL-7R expression in B-cell development and B-cell acute lymphoblastic leukemia
PMID: 38058209
3
PELI2 inhibits colorectal cancer development and progression through MAPK signaling pathway suppression
PMID: 40500708
4
PELI2 Thr257Ile variant causes constitutive NLRP3 inflammasome activation in ocular pterygium-digital keloid dysplasia
PMID: 36776133
5
PELI2 upregulation contributes to LPS-induced acute lung injury; miR-802 provides protective effects by downregulating PELI2
PMID: 31696241
6
PELI2 promotes gastric cancer growth and lymphatic metastasis by regulating vascular endothelial growth factor C
PMID: 30971501
Disease Associationsⓘ20
alcohol drinkingOpen Targets
0.44Moderate
mathematical abilityOpen Targets
0.41Moderate
smoking initiationOpen Targets
0.35Weak
ulcerative colitisOpen Targets
0.29Weak
exostosisOpen Targets
0.28Weak
asthmaOpen Targets
0.28Weak
multinodular goiterOpen Targets
0.27Weak
rhabdomyolysisOpen Targets
0.24Weak
brain aneurysmOpen Targets
0.22Weak
diabetic ketoacidosisOpen Targets
0.20Weak
thyroid cancerOpen Targets
0.20Weak
colorectal carcinomaOpen Targets
0.07Suggestive
neoplasmOpen Targets
0.04Suggestive
Miyoshi myopathyOpen Targets
0.04Suggestive
Varicose veinsOpen Targets
0.04Suggestive
Abnormality of the gastrointestinal tractOpen Targets
0.04Suggestive
immunodeficiency 89 and autoimmunityOpen Targets
0.03Suggestive
hyper-IgE recurrent infection syndrome 5, autosomal recessiveOpen Targets
0.03Suggestive
lymphatic system diseaseOpen Targets
0.03Suggestive
vein disorderOpen Targets
0.03Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
IRAK1Protein interaction100%IRAK4Protein interaction96%TRAF6Protein interaction95%UBE2NProtein interaction73%MYDGFShared pathway25%C18orf32Shared pathway25%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
61%
Lung
32%
Brain
26%
Heart
15%
Liver
7%
Gene Interaction Network
Click a node to explore
PELI2IRAK1IRAK4TRAF6UBE2NMYDGFC18orf32
PROTEIN STRUCTURE
Preparing viewer…
PDB3EGA · 1.80 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.84LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.58 [0.41–0.84]
RankingsWhere PELI2 stands among ~20K protein-coding genes
  • #11,206of 20,598
    Most Researched34
  • #7,231of 17,882
    Most Constrained (LOEUF)0.84
Genes detectedPELI2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
PELI2 is a negative regulator of STING signaling that is dynamically repressed during viral infection.
PMID: 38917796
Mol Cell · 2024
1.00
2
PMID: 38058209
Haematologica · 2024
0.90
3
PELI2 inhibits colorectal cancer development through MAPK signaling pathway.
PMID: 40500708
Mol Med · 2025
0.80
4
A Pellino-2 variant is associated with constitutive NLRP3 inflammasome activation in a family with ocular pterygium-digital keloid dysplasia.
PMID: 36776133
FEBS Lett · 2023
0.70
5
Identification of a prognostic 28-gene expression signature for gastric cancer with lymphatic metastasis.
PMID: 30971501
Biosci Rep · 2019
0.60