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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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BTN3A3
butyrophilin subfamily 3 member A3
Chromosome 6 · 6p22.2
NCBI Gene: 10384Ensembl: ENSG00000111801.17HGNC: HGNC:1140UniProt: O00478
28PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingT cell mediated immunityplasma membranemembraneneoplasmovarian cancernon-small cell lung carcinomadepressive disorder
✦AI Summary

BTN3A3 is a butyrophilin family member that functions as a membrane protein with dual roles in immune regulation and tumor suppression. Primarily, BTN3A3 participates in γδ T cell activation through heteromerization with BTN3A1 and BTN2A1 to form phosphoantigen-induced receptor complexes that trigger γδ TCR signaling 1. The protein is transcriptionally regulated by NLRC5, a transcriptional regulator of MHC class I genes, linking it to non-conventional T cell responses against metabolically abnormal cells 2. Beyond immunity, BTN3A3 exhibits antiviral properties specifically against avian influenza viruses by inhibiting viral RNA replication at early infection stages, with viral evasion determined by specific nucleoprotein residues 3. In cancer biology, BTN3A3 functions as a tumor suppressor in clear cell renal cell carcinoma, suppressing proliferation and migration through RPS3A interaction and ROS/MAPK pathway modulation 4. Clinically, BTN3A3 variants are implicated in generalized pustular psoriasis (GPP), an autoinflammatory keratinization disease, where loss of function activates NF-κB signaling and IL-36 inflammatory axis disruption 56. BTN3A3 is also identified as a potential drug target for psychiatric disorders including schizophrenia and bipolar disorder 7, and as a therapeutic target for chr6 kidney disease progression 8.

Sources cited
1
BTN3A3 forms heteromers with BTN3A1 that enhance γδ TCR activation through phosphoantigen-induced complex stabilization
PMID: 40334665
2
BTN3A3 is transcriptionally regulated by NLRC5 and regulates Vγ9Vδ2 T cell responses against metabolically abnormal cells
PMID: 39035010
3
BTN3A3 inhibits avian influenza virus replication at early viral life cycle stages by targeting nucleoprotein residues
PMID: 37380775
4
BTN3A3 acts as tumor suppressor in ccRCC by interacting with RPS3A to regulate ROS/MAPK signaling and inhibit cell proliferation/migration
PMID: 37806541
5
BTN3A3 variants are associated with generalized pustular psoriasis; loss of function promotes NF-κB activation and IL-36 axis disruption
PMID: 36804966
6
BTN3A3 is identified as GPP-related gene involved in excessive innate immune pathway activation and autoinflammation
PMID: 39698752
7
BTN3A3 is identified as genetically supported drug target for schizophrenia and bipolar disorder
PMID: 37418754
8
BTN3A3 is identified as potential therapeutic target for chronic kidney disease through proteome-transcriptome integration analysis
PMID: 38898508
Disease Associationsⓘ20
neoplasmOpen Targets
0.08Suggestive
ovarian cancerOpen Targets
0.08Suggestive
non-small cell lung carcinomaOpen Targets
0.06Suggestive
depressive disorderOpen Targets
0.05Suggestive
astrocytomaOpen Targets
0.02Suggestive
clear cell renal carcinomaOpen Targets
0.02Suggestive
lung carcinomaOpen Targets
0.02Suggestive
Oral ulcerOpen Targets
0.01Suggestive
Crohn's diseaseOpen Targets
0.01Suggestive
breast cancerOpen Targets
0.01Suggestive
cervical cancerOpen Targets
0.01Suggestive
ovarian carcinomaOpen Targets
0.01Suggestive
juvenile dermatomyositisOpen Targets
0.01Suggestive
Sjogren syndromeOpen Targets
0.01Suggestive
type 1 diabetes mellitusOpen Targets
0.01Suggestive
hepatocellular carcinomaOpen Targets
0.01Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.01Suggestive
rheumatoid arthritisOpen Targets
0.01Suggestive
generalized pustular psoriasisOpen Targets
0.01Suggestive
cancerOpen Targets
0.01Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
XDHProtein interaction97%PLIN2Protein interaction92%CLEC4GProtein interaction82%MFGE8Protein interaction79%BTN3A2Protein interaction77%BTN3A1Protein interaction75%
Tissue Expression6 tissues
Lung
100%
Ovary
58%
Liver
49%
Heart
47%
Bone Marrow
38%
Brain
16%
Gene Interaction Network
Click a node to explore
BTN3A3XDHPLIN2CLEC4GMFGE8BTN3A2BTN3A1
PROTEIN STRUCTURE
Preparing viewer…
PDB6J0G · 1.60 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.13LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.87 [0.68–1.13]
RankingsWhere BTN3A3 stands among ~20K protein-coding genes
  • #12,317of 20,598
    Most Researched28
  • #11,722of 17,882
    Most Constrained (LOEUF)1.13
Genes detectedBTN3A3
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Identification of novel therapeutic targets for chronic kidney disease and kidney function by integrating multi-omics proteome with transcriptome.
PMID: 38898508
Genome Med · 2024
1.00
2
BTN3A3 evasion promotes the zoonotic potential of influenza A viruses.
PMID: 37380775
Nature · 2023
0.90
3
Phosphoantigen-induced inside-out stabilization of butyrophilin receptor complexes drives dimerization-dependent γδ TCR activation.
PMID: 40334665
Immunity · 2025
0.80
4
Mendelian Randomization Using the Druggable Genome Reveals Genetically Supported Drug Targets for Psychiatric Disorders.
PMID: 37418754
Schizophr Bull · 2023
0.70
5
A Ménage à trois: NLRC5, immunity, and metabolism.
PMID: 39035010
Front Immunol · 2024
0.60