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GeneE
10 sources retrieved Β· Most recent: April 2026 Β· Index updated 15 days ago
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ADGRB3
adhesion G protein-coupled receptor B3
Chromosome 6 Β· 6q12-q13
NCBI Gene: 577Ensembl: ENSG00000135298.15HGNC: HGNC:945UniProt: O60242
33PubMed Papers
20Diseases
0Drugs
2Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedReceptor
DATA QUALITY
βœ“ Experimental GO Evidenceβœ“ Swiss-Prot Reviewed
plasma membraneprotein bindingmyoblast fusionpostsynapseShort statureidiopathic pulmonary fibrosisPainbreast benign neoplasm
✦AI Summary

ADGRB3 (adhesion G protein-coupled receptor B3) is an adhesion GPCR primarily expressed in the brain that regulates synaptogenesis and dendritic spine formation through interaction with ELMO1 and RAC1 signaling pathways 1. The receptor also promotes myoblast fusion via ELMO/DOCK1 mechanisms. Beyond its classical neurological functions, ADGRB3 plays unexpected roles in immune regulation and cancer biology. In T cells, ADGRB3 expression is maintained by the NM II-NKX3-2 pathway and modulates filamentous actin organization to control traction force and cytotoxicity against tumors 2. Loss-of-function mutations in ADGRB3 are associated with neurodevelopmental disorders; biallelic ADGRB3 duplications cause intellectual disability, cerebellar atrophy, and behavioral abnormalities 3. ADGRB3-deficient mice exhibit reduced brain and body weights with social interaction deficits 4. In cancer contexts, ADGRB3 alterations correlate with improved immunotherapy outcomes in both extensive-stage small cell lung cancer 5 and advanced biliary duct cancer 6, suggesting therapeutic potential. ADGRB3 expression also impacts overall survival in endometrial cancer 7. These findings establish ADGRB3 as a multifunctional receptor linking synaptic development, immune cell mechanics, and cancer immunotherapy responses.

Sources cited
1
ADGRB3 (formerly BAI3) is classified as an adhesion GPCR and regulates multiple biological processes
PMID: 25713288
2
ADGRB3 is regulated by the NM II-NKX3-2 pathway in T cells and controls F-actin organization and traction force, affecting T cell cytotoxicity against tumors
PMID: 39485850
3
Biallelic ADGRB3 duplications cause intellectual disability, ataxia, cerebellar involvement, and behavioral disorder
PMID: 30659260
4
ADGRB3-deficient mice lack full-length protein expression, show reduced brain and body weights, and demonstrate social interaction deficits
PMID: 37337931
5
ADGRB3 alterations are more frequently observed in long-term survivors of extensive-stage small cell lung cancer receiving chemoimmunotherapy
PMID: 39151386
6
ADGRB3 mutations are enriched in advanced biliary duct cancer patients responding to durvalumab plus chemotherapy and correlate with superior survival
PMID: 39235609
7
High ADGRB3 expression correlates with shorter overall survival in uterine corpus endometrial carcinoma patients
PMID: 35413679
Disease Associationsβ“˜20
Short statureOpen Targets
0.32Weak
idiopathic pulmonary fibrosisOpen Targets
0.29Weak
PainOpen Targets
0.29Weak
breast benign neoplasmOpen Targets
0.29Weak
insomniaOpen Targets
0.27Weak
pneumonitisOpen Targets
0.27Weak
nephritisOpen Targets
0.25Weak
NephropathyOpen Targets
0.25Weak
disturbances of sensation of smell and tasteOpen Targets
0.24Weak
asthmaOpen Targets
0.23Weak
restless legs syndromeOpen Targets
0.20Weak
Alzheimer diseaseOpen Targets
0.20Weak
pneumothoraxOpen Targets
0.19Weak
breast diseaseOpen Targets
0.19Weak
preeclampsiaOpen Targets
0.18Weak
pleural empyemaOpen Targets
0.18Weak
skin woundOpen Targets
0.17Weak
ankylosing spondylitisOpen Targets
0.16Weak
plasma fibronectin deficiencyOpen Targets
0.06Suggestive
isolated asymptomatic elevation of creatine phosphokinaseOpen Targets
0.05Suggestive
Pathogenic Variants2
NM_001704.3(ADGRB3):c.2294T>C (p.Val765Ala)Likely pathogenic
Short stature
β˜†β˜†β˜†β˜†2001β†’ Residue 765
NM_001704.3(ADGRB3):c.4298G>A (p.Arg1433Lys)Likely pathogenic
Short stature
β˜†β˜†β˜†β˜†2001β†’ Residue 1433
View on ClinVar β†—
Related Genes
RANGAP1Protein interaction100%C1QL3Protein interaction83%C1QL1Protein interaction80%GRM8Protein interaction73%ELMO1Protein interaction72%ADGRD2Co-mentioned in literature67%
Tissue Expression6 tissues
Brain
100%
Heart
25%
Lung
8%
Ovary
8%
Liver
6%
Bone Marrow
0%
Gene Interaction Network
Click a node to explore
ADGRB3RANGAP1C1QL3C1QL1GRM8ELMO1ADGRD2
PROTEIN STRUCTURE
Preparing viewer…
PDB4DLO Β· 2.30 Γ… Β· X-ray
View on RCSB β†—
Constraintβ“˜
LOEUFβ“˜
0.25Highly Constrained
pLIβ“˜
1.00Intolerant
Observed/Expected LoF0.18 [0.13–0.25]
RankingsWhere ADGRB3 stands among ~20K protein-coding genes
  • #11,281of 20,598
    Most Researched33
  • #4,496of 5,498
    Most Pathogenic Variants2
  • #744of 17,882
    Most Constrained (LOEUF)0.25 Β· top 5%
Genes detectedADGRB3
Sources retrieved10 papers
Response timeβ€”
πŸ“„ Sources
10β–Ό
1
International Union of Basic and Clinical Pharmacology. XCIV. Adhesion G protein-coupled receptors.
PMID: 25713288
Pharmacol Rev Β· 2015
1.00
2
Suppression of non-muscle myosin II boosts T cell cytotoxicity against tumors.
PMID: 39485850
Sci Adv Β· 2024
0.90
3
Biallelic intragenic duplication in ADGRB3 (BAI3) gene associated with intellectual disability, cerebellar atrophy, and behavioral disorder.
PMID: 30659260
Eur J Hum Genet Β· 2019
0.80
4
A correlation study of adhesion G protein-coupled receptors as potential therapeutic targets in Uterine Corpus Endometrial cancer.
PMID: 35413679
Int Immunopharmacol Β· 2022
0.70
5
Generation and initial characterization of mice lacking full-length BAI3 (ADGRB3) expression.
PMID: 37337931
Basic Clin Pharmacol Toxicol Β· 2023
0.60