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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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BPIFA1
BPI fold containing family A member 1
Chromosome 20 · 20q11.21
NCBI Gene: 51297Ensembl: ENSG00000198183.14HGNC: HGNC:15749UniProt: Q9NP55
108PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingGO:0005615extracellular regionimmune response in nasopharyngeal-associated lymphoid tissueneurodegenerative diseaseParkinson diseaselysosomal storage diseaseAlzheimer disease
✦AI Summary

BPIFA1 (BPI fold containing family A member 1) is a secretory lipid-binding protein with primary specificity for surfactant phospholipid DPPC 1 that functions as a key component of innate immune defense in mucosal tissues. Mechanistically, BPIFA1 reduces surface tension in airway secretions and inhibits biofilm formation by pathogenic Gram-negative bacteria including Pseudomonas aeruginosa and Klebsiella pneumoniae 23. Additionally, BPIFA1 negatively regulates proteolytic cleavage of SCNN1G, thereby preventing inappropriate ENaC activation and maintaining airway surface liquid homeostasis 45. Gene expression marks epithelial differentiation during mucociliary development 6, and BPIFA1 expression is documented across upper airway tissues including nasal cavity, sinuses, and middle ear 78. Disease relevance is significant: reduced BPIFA1 expression associates with asthma severity and greater eosinophilic inflammation, particularly in patients carrying the rs750064 CC genotype 9. BPIFA1 regulates anti-inflammatory macrophage polarization and suppresses NF-κB signaling in periodontitis 10. Novel antimicrobial peptides derived from BPIFA1 demonstrate antifungal activity against Candida albicans 11. Clinically, salivary BPIFA1 levels increase during head and neck cancer radiotherapy, correlating with mucositis severity 12. Emerging evidence suggests BPIFA1 as an immunotherapeutic target for inflammatory airway diseases.

Sources cited
1
BPIFA1 (BPI fold containing family A member 1) is a secretory lipid-binding protein with primary specificity for surfactant phospholipid DPPC that functions as a key component of innate immune defense in mucosal tissues.
PMID: 25223608
2
Gene expression marks epithelial differentiation during mucociliary development , and BPIFA1 expression is documented across upper airway tissues including nasal cavity, sinuses, and middle ear , .
PMID: 36519134
3
Disease relevance is significant: reduced BPIFA1 expression associates with asthma severity and greater eosinophilic inflammation, particularly in patients carrying the rs750064 CC genotype .
PMID: 30996135
4
BPIFA1 regulates anti-inflammatory macrophage polarization and suppresses NF-κB signaling in periodontitis .
PMID: 39970751
5
Novel antimicrobial peptides derived from BPIFA1 demonstrate antifungal activity against Candida albicans .
PMID: 35201913
6
Clinically, salivary BPIFA1 levels increase during head and neck cancer radiotherapy, correlating with mucositis severity .
PMID: 25482547
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.38Weak
Alzheimer diseaseOpen Targets
0.31Weak
lysosomal storage diseaseOpen Targets
0.31Weak
multiple sclerosisOpen Targets
0.31Weak
Parkinson diseaseOpen Targets
0.31Weak
nasopharyngeal carcinomaOpen Targets
0.11Weak
non-small cell lung carcinomaOpen Targets
0.10Weak
cystic fibrosisOpen Targets
0.10Weak
neoplasmOpen Targets
0.09Suggestive
Immunodeficiency due to a late component of complements deficiencyOpen Targets
0.09Suggestive
chronic obstructive pulmonary diseaseOpen Targets
0.09Suggestive
periodontitisOpen Targets
0.08Suggestive
Chronic mucocutaneous candidosisOpen Targets
0.08Suggestive
immunodeficiency 28Open Targets
0.07Suggestive
Mendelian susceptibility to mycobacterial diseases due to complete IFNgammaR2 deficiencyOpen Targets
0.07Suggestive
type 2 diabetes mellitusOpen Targets
0.07Suggestive
Autosomal recessive hyper-IgE syndromeOpen Targets
0.07Suggestive
varicella, severe recurrentOpen Targets
0.07Suggestive
immunodeficiency 31BOpen Targets
0.07Suggestive
Susceptibility to viral and mycobacterial infectionsOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
BPIFB1Protein interaction89%BPIFB4Protein interaction86%COX7B2Protein interaction83%BPIFB2Protein interaction82%BPIFA2Protein interaction78%CETPProtein interaction76%
Tissue Expression6 tissues
Lung
100%
Bone Marrow
7%
Liver
0%
Brain
0%
Heart
0%
Ovary
0%
Gene Interaction Network
Click a node to explore
BPIFA1BPIFB1BPIFB4COX7B2BPIFB2BPIFA2CETP
PROTEIN STRUCTURE
Preparing viewer…
PDB4KEG · 2.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.35LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF0.96 [0.70–1.35]
RankingsWhere BPIFA1 stands among ~20K protein-coding genes
  • #4,396of 20,598
    Most Researched108 · top quartile
  • #14,162of 17,882
    Most Constrained (LOEUF)1.35
Genes detectedBPIFA1
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
A protein interaction landscape of breast cancer.
PMID: 34591612
Science · 2021
1.00
2
BPIFA1 Gene Expression in the Human Middle Ear Mucosa.
PMID: 29360089
J Int Adv Otol · 2017
0.90
3
The Role of BPIFA1 in Upper Airway Microbial Infections and Correlated Diseases.
PMID: 30255091
Biomed Res Int · 2018
0.80
4
BPIFA1 is a secreted biomarker of differentiating human airway epithelium.
PMID: 36519134
Front Cell Infect Microbiol · 2022
0.70
5
BPIFA1 inhibits periodontitis by regulating the NF-κB/IκB signaling pathway and macrophage M1/M2 polarization.
PMID: 39970751
Arch Oral Biol · 2025
0.60