ArticleEBioMedicine2020
Lung immunoglobulin A immunity dysregulation in cystic fibrosis.
Article in EBioMedicine, 2020. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 28 papers.
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Who cites it
28 citing papers in PubMed, 35 citations in OpenAlex.
- Respiratory mucosal immunity: Biological functions, diseases, prevention and therapy.Genes & diseases · 2026Review
- Mitochondria Meet the Lung Microbiome: A Bidirectional Dialogue in Inflammation and Respiratory Diseases.Biomedicines · 2026Review
- Crosstalk between the microbiome and the mucosal immunoglobulin A system in the lung, in health and disease.Frontiers in cellular and infection microbiology · 2026Review
- Airway mucus in infection.Frontiers in physiology · 2026Review
- Evidence for Interleukin-17C governing interleukin-17A pathogenicity and promoting asthma endotype switching in bronchiectasis.Nature communications · 2025Article
- Immunoglobulin A mucosal immunity in people with cystic fibrosis shortly after initiation of highly effective modulator therapy.ERJ open research · 2025Article
- Impaired IgA mucosal immunity following lung transplantation: a potential trigger for bronchiolitis obliterans syndrome.The European respiratory journal · 2025Article
- Development of a cyst-targeted therapy for polycystic kidney disease using an antagonistic dimeric IgA monoclonal antibody against cMET.Cell reports. Medicine · 2025Article
- Polymeric immunoglobulin receptor (pIgR) in cancer progression: a critical role and potential therapeutic target.Apoptosis : an international journal on programmed cell death · 2025Review
- LpxR and PagL expression in live attenuated auxotrophicFrontiers in cellular and infection microbiology · 2025Article
- Airway epithelium damage in acute respiratory distress syndrome.Critical care (London, England) · 2024Article
- Evaluation of anti-vector immune responses to adenovirus-mediated lung gene therapy and modulation by αCD20.Molecular therapy. Methods & clinical development · 2024Article
- The memory of airway epithelium damage in smokers and COPD patients.Life science alliance · 2024Article
- IgA-producing B cells in lung homeostasis and disease.Frontiers in immunology · 2023Review
- A new hope? Possibilities of therapeutic IgA antibodies in the treatment of inflammatory lung diseases.Frontiers in immunology · 2023Review
- A spatially resolved atlas of the human lung characterizes a gland-associated immune niche.Nature genetics · 2023Article
- The Impact of Highly Effective Modulator Therapy on Cystic Fibrosis Microbiology and Inflammation.Clinics in chest medicine · 2022Review
- Review
- Immune Response to Biofilm Growing PulmonaryBiomedicines · 2022Review
- Review
Corrections and comments
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Authors and funding
18 authors at 6 institutions in 3 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
backgroundIn cystic fibrosis (CF), recurrent infections suggest impaired mucosal immunity but whether production of secretory immunoglobulin A (S-IgA) is impaired remains elusive. S-IgA is generated following polymeric immunoglobulin receptor (pIgR)-mediated transepithelial transport of dimeric (d-)IgA and represents a major defence through neutralisation of inhaled pathogens like Pseudomonas aeruginosa (Pa).
methodsHuman lung tissue (n = 74), human sputum (n = 118), primary human bronchial epithelial cells (HBEC) (cultured in air-liquid interface) (n = 19) and mouse lung tissue and bronchoalveolar lavage were studied for pIgR expression, IgA secretion and regulation.
findingsIncreased epithelial pIgR immunostaining was observed in CF lung explants, associated with more IgA-producing plasma cells, sputum and serum IgA, especially Pa-specific IgA. In contrast, pIgR and IgA transport were downregulated in F508del mice, CFTR-inhibited HBEC, and CF HBEC. Moreover, the unfolded protein response (UPR) due to F508del mutation, inhibited IgA transport in Calu-3 cells. Conversely, pIgR expression and IgA secretion were strongly upregulated following Pa lung infection in control and F508del mice, through an inflammatory host response involving interleukin-17.
interpretationA complex regulation of IgA secretion occurs in the CF lung, UPR induced by CFTR mutation/dysfunction inhibiting d-IgA transcytosis, and Pa infection unexpectedly unleashing this secretory defence mechanism.
fundingThis work was supported by the Forton's grant of the King Baudouin's Foundation, Belgium, the Fondazione Ricerca Fibrosi Cistica, Italy, and the Fonds National de la Recherche Scientifique, Belgium.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.