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The Host-Pathogen Interaction in Cystic Fibrosis

A special issue of Pathogens (ISSN 2076-0817).

Deadline for manuscript submissions: closed (31 January 2026) | Viewed by 1432

Special Issue Editors


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Guest Editor
Department of Diagnostics and Public Health, University of Verona, 37134 Verona, VR, Italy
Interests: cystic fibrosis; respiratory infections; bacterial pathogens
Special Issues, Collections and Topics in MDPI journals

E-Mail Website
Guest Editor
Department of Diagnostics and Public Health, University of Verona, 37134 Verona, VR, Italy
Interests: cystic fibrosis; respiratory infections; oral infections

E-Mail Website
Guest Editor
Infections and Cystic Fibrosis Unit, Division of Immunology, Transplantation and Infectious Diseases, IRCCS San Raffaele Scientific Institute, Milan, Italy
Interests: microbial genomics; host-pathogen interactions; multi-layer omics
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

This Special Issue will focus on host–pathogen interactions with regard to respiratory infections in cystic fibrosis (CF).

CF lung disease is a complex condition wherein pathogenic microorganisms and defective immune system cooperate in developing a constant cycle of infection and inflammation. Investigating how the CF host immune system interacts with these organisms is crucial to understand the pathogenesis of the pulmonary disease and develop new approaches for its treatment.

This Special Issue welcomes manuscripts providing advances in elucidating the microbial complexity present in the airways of individuals with CF and their interaction with the innate and adaptive immune system; proposing targeted therapies to fight infection and to reduce the aberrant inflammatory response that characterizes CF; and presenting the effect of CFTR modulators on microbial infection and the inflammatory process, in light of the recent adoption of these therapeutics that correct and potentiate some classes of mutations of the CFTR.

We look forward to your valuable contributions that will promote further developments in this field.

Thank you for your collaboration.

Dr. Angela Sandri
Dr. Caterina Signoretto
Dr. Laura Veschetti
Guest Editors

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Keywords

  • cystic fibrosis
  • respiratory infection
  • host–pathogen interaction
  • CFTR
  • immune system

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Published Papers (1 paper)

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Research

14 pages, 1909 KB  
Article
Role of S1PR1 in Modulating Airway Epithelial Responses to Pseudomonas aeruginosa in Cystic Fibrosis
by Cristina Cigana, Claudia Caslini, Alessandro Migliara, Beatriz Alcala’-Franco, Laura Veschetti, Nicola Ivan Lorè, Angelo Lombardo and Alessandra Bragonzi
Pathogens 2025, 14(11), 1146; https://doi.org/10.3390/pathogens14111146 - 12 Nov 2025
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Abstract
Background: Pseudomonas aeruginosa infection is a major driver of morbidity and mortality in cystic fibrosis (CF), yet disease severity varies widely among people with CF (pwCF). This clinical heterogeneity suggests the involvement of host genetic modifiers beyond CFTR. We previously identified [...] Read more.
Background: Pseudomonas aeruginosa infection is a major driver of morbidity and mortality in cystic fibrosis (CF), yet disease severity varies widely among people with CF (pwCF). This clinical heterogeneity suggests the involvement of host genetic modifiers beyond CFTR. We previously identified sphingosine 1-phosphate receptor 1 (S1PR1) as a candidate gene associated with susceptibility to P. aeruginosa. Here, we investigated its role in modulating airway epithelial responses to infection. Methods: Using CRISPR/Cas9, we generated S1PR1-knockout bronchial epithelial cells with (IB3-1) and without (C38) CFTR mutations. We assessed cell viability, cytotoxicity, and interleukin-8 secretion following exposure to P. aeruginosa exoproducts. S1PR1 protein expression was evaluated in lung tissue from pwCF and non-CF individuals using immunohistochemistry. Results: S1PR1-mutant cells produced truncated, non-functional peptides. In CFTR-mutant cells, S1PR1 loss reduced viability, increased cytotoxicity, and significantly enhanced interleukin-8 production in response to P. aeruginosa exoproducts. These effects were not observed in CFTR-competent cells. Notably, S1PR1 protein levels were markedly lower in lung tissue from pwCF compared to non-CF individuals. Conclusions: S1PR1 deficiency exacerbates epithelial damage and inflammatory responses to P. aeruginosa in CF models. These findings highlight S1PR1 as a potential contributor to infection severity and a promising target for therapeutic strategies in pwCF. Full article
(This article belongs to the Special Issue The Host-Pathogen Interaction in Cystic Fibrosis)
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