ArticleHistochemistry and cell biology2012
BPIFB1 (LPLUNC1) is upregulated in cystic fibrosis lung disease.
Article in Histochemistry and cell biology, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 34 citations in OpenAlex.
- Single-nucleus RNA sequencing identifies conserved structural cell programs in allergic lung inflammation.The journal of allergy and clinical immunology. Global · 2026Article
- FGFR signaling establishes spatial gradients of secretory cell identities along the airway proximal-distal axis.Nature communications · 2026Article
- The single-cell transcriptional landscape of the pediatric cystic fibrosis lung from minimally invasive respiratory specimens.Scientific reports · 2026Article
- Crosstalk betweenFrontiers in immunology · 2026Article
- Proteomic networks and related genetic variants associated with smoking and chronic obstructive pulmonary disease.BMC genomics · 2024Article
- Investigating adverse genomic and regulatory changes caused by replacement of the full-lengthMolecular therapy. Nucleic acids · 2024Article
- BPIFB1 loss alters airway mucus properties and diminishes mucociliary clearance.American journal of physiology. Lung cellular and molecular physiology · 2023Article
- Intense light-elicited alveolar type 2-specific circadian PER2 protects from bacterial lung injury via BPIFB1.American journal of physiology. Lung cellular and molecular physiology · 2022Article
- BPIFA1 is a secreted biomarker of differentiating human airway epithelium.Frontiers in cellular and infection microbiology · 2022Article
- Nintedanib induces gene expression changes in the lung of induced-rheumatoid arthritis-associated interstitial lung disease mice.PloS one · 2022Article
- Evaluation of polyhexamethylene guanidine-induced lung injuries by chest CT, pathologic examination, and RNA sequencing in a rat model.Scientific reports · 2021Article
- Molecular biology of BPIFB1 and its advances in disease.Annals of translational medicine · 2020Review
- Article
- Attached stratified mucus separates bacteria from the epithelial cells in COPD lungs.JCI insight · 2018Article
- Presence of BPIFB1 in saliva from non-obese diabetic mice.Odontology · 2018Article
- Association of innate defense proteins BPIFA1 and BPIFB1 with disease severity in COPD.International journal of chronic obstructive pulmonary disease · 2018Article
- Identification ofGenetics · 2017Article
- Identification and quantification of novel RNA isoforms in horn cancer of Bos indicus by comprehensive RNA-Seq.3 Biotech · 2016Article
- Low-frequency coding variants at 6p21.33 and 20q11.21 are associated with lung cancer risk in Chinese populations.American journal of human genetics · 2015Article
- Short palate, lung, and nasal epithelial clone-1 is a tightly regulated airway sensor in innate and adaptive immunity.American journal of respiratory cell and molecular biology · 2013Article
Corrections and comments
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Authors and funding
11 authors at 7 institutions in 5 countries.
Funding
Abstract
Although the biology the PLUNC (recently renamed BPI fold, BPIF) family of secreted proteins is poorly understood, multiple array based studies have suggested that some are differentially expressed in lung diseases. We have examined the expression of BPIFB1 (LPLUNC1), the prototypic two-domain containing family member, in lungs from CF patients and in mouse models of CF lung disease. BPIFB1 was localized in CF lung samples along with BPIFA1, MUC5AC, CD68 and NE and directly compared to histologically normal lung tissues and that of bacterial pneumonia. We generated novel antibodies to mouse BPIF proteins to conduct similar studies on ENaC transgenic (ENaC-Tg) mice, a model for CF-like lung disease. Small airways in CF demonstrated marked epithelial staining of BPIFB1 in goblet cells but staining was absent from alveolar regions. BPIFA1 and BPIFB1 were not co-localised in the diseased lungs. In ENaC-Tg mice there was strong staining of both proteins in the airways and luminal contents. This was most marked for BPIFB1 and was noted within 2 weeks of birth. The two proteins were present in distinct cells within epithelium. BPIFB1 was readily detected in BAL from ENaC-Tg mice but was absent from wild-type mice. Alterations in the expression of BPIF proteins is associated with CF lung disease in humans and mice. It is unclear if this elevation of protein production, which results from phenotypic alteration of the cells within the diseased epithelium, plays a role in the pathogenesis of the disease.
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