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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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ADGRE2
adhesion G protein-coupled receptor E2
Chromosome 19 · 19p13.12
NCBI Gene: 30817Ensembl: ENSG00000127507.18HGNC: HGNC:3337UniProt: A0JNV7
54PubMed Papers
21Diseases
0Drugs
1Pathogenic Variants
FUNCTIONAL ROLE
Receptor
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
granulocyte chemotaxiscell adhesionprotein bindingcell migrationvibratory urticariaautosomal dominant vibratory urticariaGenetic visceral malformation of the liver, biliary tract, pancreas or spleenneoplasm
✦AI Summary

ADGRE2 (adhesion G protein-coupled receptor E2) is a cell surface receptor that functions as a key regulator of immune cell activation and leukemic progression. The receptor binds chondroitin sulfate moieties on glycosaminoglycan chains and promotes granulocyte chemotaxis, degranulation, and adhesion 1. In macrophages, ADGRE2 stimulates release of inflammatory cytokines including IL-8 and TNF, while in mast cells it regulates degranulation through G protein-coupled signaling 2. ADGRE2 represents an antibody-independent mast cell receptor with emerging roles in allergic disease mechanisms 3. In acute myeloid leukemia (AML), ADGRE2 is highly expressed in leukemic stem cells and associates with poor patient outcomes 4. Mechanistically, ADGRE2 activates phospholipase Cβ/protein kinase C/MEK/ERK signaling to enhance AP1-driven expression of DUSP1, a protein phosphatase that maintains proteostasis via DNAJB1-HSP70 interaction 4. Combined MEK, AP1, and DUSP1 inhibition shows robust therapeutic efficacy in AML xenograft models. Clinically, dual-targeting approaches combining attenuated ADGRE2-CAR with CLEC12A costimulation selectively eliminate AML while minimizing hematopoietic stem cell toxicity 5. Additionally, common ADGRE2 repeat alleles exhibit high blood mosaicism rates, demonstrating somatic instability during aging 6.

Sources cited
1
ADGRE2 (EMR2) is an adhesion GPCR that binds chondroitin sulfate and signals through G proteins
PMID: 25713288
2
ADGRE2 is expressed on immune cells including granulocytes and macrophages and modulates immune cell function
PMID: 27832495
3
ADGRE2 is highly expressed in AML leukemic stem cells, associates with poor outcomes, and activates MEK/ERK/AP1/DUSP1 signaling to maintain proteostasis
PMID: 39082681
4
ADGRE2 can be targeted by CAR-T cell therapy in combination with CLEC12A to selectively eliminate AML while sparing normal hematopoietic stem cells
PMID: 37802054
5
ADGRE2 contains DNA repeat elements that show high rates of somatic length mosaicism in blood
PMID: 41501457
6
ADGRE2 is an antibody-independent mast cell receptor involved in allergic disease mechanisms
PMID: 32727338
Disease Associationsⓘ21
vibratory urticariaOpen Targets
0.59Moderate
autosomal dominant vibratory urticariaOpen Targets
0.41Moderate
Genetic visceral malformation of the liver, biliary tract, pancreas or spleenOpen Targets
0.14Weak
neoplasmOpen Targets
0.11Weak
glioblastoma multiformeOpen Targets
0.11Weak
acute myeloid leukemiaOpen Targets
0.10Weak
hepatocellular carcinomaOpen Targets
0.08Suggestive
intrahepatic cholangiocarcinomaOpen Targets
0.08Suggestive
ovarian cancerOpen Targets
0.07Suggestive
glioblastomaOpen Targets
0.07Suggestive
X-linked severe congenital neutropeniaOpen Targets
0.06Suggestive
gastric cancerOpen Targets
0.06Suggestive
Immunodeficiency due to interleukin-1 receptor-associated kinase-4 deficiencyOpen Targets
0.06Suggestive
Recurrent infection due to specific granule deficiencyOpen Targets
0.06Suggestive
gliomaOpen Targets
0.05Suggestive
neutropenia, severe congenital, 1, autosomal dominantOpen Targets
0.05Suggestive
nonimmune chronic idiopathic neutropenia of adultsOpen Targets
0.05Suggestive
arthritisOpen Targets
0.05Suggestive
autosomal recessive severe congenital neutropenia due to CSF3R deficiencyOpen Targets
0.05Suggestive
transient myeloproliferative syndromeOpen Targets
0.05Suggestive
Vibratory urticariaUniProt
Pathogenic Variants1
NM_013447.4(ADGRE2):c.1276C>T (p.Gln426Ter)Likely pathogenic
Vibratory urticaria
★☆☆☆2021→ Residue 426
View on ClinVar ↗
Related Genes
CD55Protein interaction100%LPAR1Protein interaction80%CD59Protein interaction76%ADGRE5Shared pathway33%SIGLEC9Shared pathway33%ADGRE1Shared pathway33%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
76%
Brain
29%
Liver
15%
Heart
6%
Ovary
3%
Gene Interaction Network
Click a node to explore
ADGRE2CD55LPAR1CD59ADGRE5SIGLEC9ADGRE1
PROTEIN STRUCTURE
Preparing viewer…
PDB2BOU · 1.90 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.03LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.85 [0.71–1.03]
RankingsWhere ADGRE2 stands among ~20K protein-coding genes
  • #8,276of 20,598
    Most Researched54
  • #5,074of 5,498
    Most Pathogenic Variants1
  • #10,148of 17,882
    Most Constrained (LOEUF)1.03
Genes detectedADGRE2
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Cooperative CAR targeting to selectively eliminate AML and minimize escape.
PMID: 37802054
Cancer Cell · 2023
1.00
2
International Union of Basic and Clinical Pharmacology. XCIV. Adhesion G protein-coupled receptors.
PMID: 25713288
Pharmacol Rev · 2015
0.90
3
Rapid identification of primary atopic disorders (PAD) by a clinical landmark-guided, upfront use of genomic sequencing.
PMID: 39381601
Allergol Select · 2024
0.80
4
Adhesion GPCR ADGRE2 Maintains Proteostasis to Promote Progression in Acute Myeloid Leukemia.
PMID: 39082681
Cancer Res · 2024
0.70
5
Vibratory Urticaria and ADGRE2.
PMID: 27406365
N Engl J Med · 2016
0.60