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Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Immunology".

Deadline for manuscript submissions: closed (31 August 2022) | Viewed by 15366

Special Issue Editors

Dr. Loic Guillot

E-Mail Website
Guest Editor
Centre de Recherche Saint‑Antoine (CRSA), INSERM UMR_S938, Sorbonne Université, 75012 Paris, France
Interests: lung; rare disease; cystic fibrosis; innate immunity; infection; genetics
Dr. Sébastien Boutin

E-Mail Website
Guest Editor
Translational Lung Research Center Heidelberg (TLRC), German Center for Lung Research (DZL), Department of Infectious Diseases, Medical Microbiology and Hygiene, Heidelberg University Hospital, Im Neuenheimer Feld 324, 69120 Heidelberg, Germany
Interests: microbiome; lung disease; cystic fibrosis; infection

Special Issue Information

Dear Colleagues,

Infections remain a major health issue worldwide and antimicrobial resistance is a serious public health threat requiring the development of alternative/complementary strategies to improve patient care. This is particularly important for patients with cystic fibrosis (CF), a rare genetic disease characterized by microbial infection including bacteria, fungi, and virus. Chronic bacterial lung infection, especially by P. aeruginosa, is a key step in the course of CF lung disease severity and is known to induce excessive inflammation in the lung and to impair airway epithelial barrier function. In fact, airways constantly exposed to pathogens through respiration have a pivotal role in the triggering and orientation of the lung immune response.

The mechanisms of microbial infection in CF are a topic of great interest, and deeper molecular dissection of the cellular host response and the microbial mechanisms involved to escape from this host response needs to be supported to discover new therapeutic strategies.

This Special Issue, “Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes”, welcomes original research and review articles addressing the regulation of the airway host response to microorganisms, the adaptation of microorganisms to the host, and the microbial dysbiosis using in vitro and in vivo model systems for CF. Studies investigating the usefulness of new model for studying these interactions, such as lung organoids/spheroids and lung-on-chip, are also encouraged.

Dr. Loic Guillot
Dr. Sébastien Boutin
Guest Editors

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • cystic fibrosis
  • immune response
  • infection
  • microorganisms

Published Papers (6 papers)

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Editorial

Jump to: Research, Review

2 pages, 201 KiB  
Editorial
Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes
by Sébastien Boutin and Loïc Guillot
Int. J. Mol. Sci. 2023, 24(9), 7766; https://doi.org/10.3390/ijms24097766 - 24 Apr 2023
Cited by 1 | Viewed by 842
Abstract
Cystic fibrosis (CF) is a rare genetic disease caused by genetic variants of the cystic fibrosis transmembrane conductance regulator (CFTR) [...] Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)

Research

Jump to: Editorial, Review

16 pages, 2880 KiB  
Article
Pseudomonas Aeruginosa Lung Infection Subverts Lymphocytic Responses through IL-23 and IL-22 Post-Transcriptional Regulation
by Bérengère Villeret, Reem Ghinnagow, Saadé Kheir, Maëlys Born-Bony, Jay K. Kolls, Ignacio Garcia-Verdugo and Jean-Michel Sallenave
Int. J. Mol. Sci. 2022, 23(15), 8427; https://doi.org/10.3390/ijms23158427 - 29 Jul 2022
Cited by 3 | Viewed by 2348
Abstract
Pseudomonas aeruginosa (P.a) is a pathogen causing significant morbidity and mortality, particularly in hospital patients undergoing ventilation and in individuals with cystic fibrosis. Although we and others have investigated mechanisms used by P.a to subvert innate immunity, relatively less is known [...] Read more.
Pseudomonas aeruginosa (P.a) is a pathogen causing significant morbidity and mortality, particularly in hospital patients undergoing ventilation and in individuals with cystic fibrosis. Although we and others have investigated mechanisms used by P.a to subvert innate immunity, relatively less is known about the potential strategies used by this bacterium to fight the adaptive immune system and, in particular, T cells. Here, using RAG KO (devoid of ‘classical’ αβ and γδ TCR T lymphocytes) and double RAG γC KO mice (devoid of T, NK and ILC cells), we demonstrate that the lymphocytic compartment is important to combat P.a (PAO1 strain). Indeed, we show that PAO1 load was increased in double RAG γC KO mice. In addition, we show that PAO1 down-regulates IL-23 and IL-22 protein accumulation in the lungs of infected mice while up-regulating their RNA production, thereby pointing towards a specific post-transcriptional regulatory mechanism not affecting other inflammatory mediators. Finally, we demonstrate that an adenovirus-mediated over-expression of IL-1, IL-23 and IL-7 induced lung neutrophil and lymphocytic influx and rescued mice against P.a-induced lethality in all WT, RAG γC KO and RAG γC KO RAG-deficient mice, suggesting that this regimen might be of value in ‘locally immunosuppressed’ individuals such as cystic fibrosis patients. Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)
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Review

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16 pages, 1944 KiB  
Review
Respiratory Infection and Inflammation in Cystic Fibrosis: A Dynamic Interplay among the Host, Microbes, and Environment for the Ages
by Christiaan Yu and Tom Kotsimbos
Int. J. Mol. Sci. 2023, 24(4), 4052; https://doi.org/10.3390/ijms24044052 - 17 Feb 2023
Cited by 5 | Viewed by 1617
Abstract
The interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung. Although [...] Read more.
The interplay between airway inflammation and infection is now recognized as a major factor in the pathobiology in cystic fibrosis (CF). A proinflammatory environment is seen throughout the CF airway resulting in classic marked and enduring neutrophilic infiltrations, irreversibly damaging the lung. Although this is seen to occur early, independent of infection, respiratory microbes arising at different timepoints in life and the world environment perpetuate this hyperinflammatory state. Several selective pressures have allowed for the CF gene to persist until today despite an early mortality. Comprehensive care systems, which have been a cornerstone of therapy for the past few decades, are now revolutionized by CF transmembrane conductance regulator (CTFR) modulators. The effects of these small-molecule agents cannot be overstated and can be seen as early as in utero. For an understanding of the future, this review looks into CF studies spanning the historical and present period. Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)
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22 pages, 745 KiB  
Review
Impact of CFTR Modulators on the Impaired Function of Phagocytes in Cystic Fibrosis Lung Disease
by Aniello Meoli, Olaf Eickmeier, Giovanna Pisi, Valentina Fainardi, Stefan Zielen and Susanna Esposito
Int. J. Mol. Sci. 2022, 23(20), 12421; https://doi.org/10.3390/ijms232012421 - 17 Oct 2022
Cited by 10 | Viewed by 2498
Abstract
Cystic fibrosis (CF), the most common genetically inherited disease in Caucasian populations, is a multi-systemic life-threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In 2012, the arrival of CFTR modulators (potentiators, correctors, amplifiers, [...] Read more.
Cystic fibrosis (CF), the most common genetically inherited disease in Caucasian populations, is a multi-systemic life-threatening autosomal recessive disorder caused by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene. In 2012, the arrival of CFTR modulators (potentiators, correctors, amplifiers, stabilizers, and read-through agents) revolutionized the therapeutic approach to CF. In this review, we examined the physiopathological mechanism of chronic dysregulated innate immune response in the lungs of CF patients with pulmonary involvement with particular reference to phagocytes, critically analyzing the role of CFTR modulators in influencing and eventually restoring their function. Our literature review highlighted that the role of CFTR in the lungs is crucial not only for the epithelial function but also for host defense, with particular reference to phagocytes. In macrophages and neutrophils, the CFTR dysfunction compromises both the intricate process of phagocytosis and the mechanisms of initiation and control of inflammation which then reverberates on the epithelial environment already burdened by the chronic colonization of pathogens leading to irreversible tissue damage. In this context, investigating the impact of CFTR modulators on phagocytic functions is therefore crucial not only for explaining the underlying mechanisms of pleiotropic effects of these molecules but also to better understand the physiopathological basis of this disease, still partly unexplored, and to develop new complementary or alternative therapeutic approaches. Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)
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22 pages, 1500 KiB  
Review
Emerging Concepts in Defective Macrophage Phagocytosis in Cystic Fibrosis
by Devi Jaganathan, Emanuela M. Bruscia and Benjamin T. Kopp
Int. J. Mol. Sci. 2022, 23(14), 7750; https://doi.org/10.3390/ijms23147750 - 13 Jul 2022
Cited by 7 | Viewed by 2746
Abstract
Cystic fibrosis (CF) is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Chronic inflammation and decline in lung function are major reasons for morbidity in CF. Mutant CFTR expressed in phagocytic cells such as macrophages contributes to persistent infection, [...] Read more.
Cystic fibrosis (CF) is caused by mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene. Chronic inflammation and decline in lung function are major reasons for morbidity in CF. Mutant CFTR expressed in phagocytic cells such as macrophages contributes to persistent infection, inflammation, and lung disease in CF. Macrophages play a central role in innate immunity by eliminating pathogenic microbes by a process called phagocytosis. Phagocytosis is required for tissue homeostasis, balancing inflammation, and crosstalk with the adaptive immune system for antigen presentation. This review focused on (1) current understandings of the signaling underlying phagocytic mechanisms; (2) existing evidence for phagocytic dysregulation in CF; and (3) the emerging role of CFTR modulators in influencing CF phagocytic function. Alterations in CF macrophages from receptor initiation to phagosome formation are linked to disease progression in CF. A deeper understanding of macrophages in the context of CFTR and phagocytosis proteins at each step of phagosome formation might contribute to the new therapeutic development of dysregulated innate immunity in CF. Therefore, the review also indicates future areas of research in the context of CFTR and macrophages. Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)
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14 pages, 675 KiB  
Review
The Effect of CFTR Modulators on Airway Infection in Cystic Fibrosis
by Caitlyn Harvey, Sinead Weldon, Stuart Elborn, Damian G. Downey and Clifford Taggart
Int. J. Mol. Sci. 2022, 23(7), 3513; https://doi.org/10.3390/ijms23073513 - 23 Mar 2022
Cited by 22 | Viewed by 3834
Abstract
The advent of Cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a critical event in the history of cystic fibrosis (CF) treatment. Unlike traditional therapies that target downstream effects of CFTR dysfunction, CFTR modulators aim to correct the underlying defect at the [...] Read more.
The advent of Cystic fibrosis transmembrane receptor (CFTR) modulators in 2012 was a critical event in the history of cystic fibrosis (CF) treatment. Unlike traditional therapies that target downstream effects of CFTR dysfunction, CFTR modulators aim to correct the underlying defect at the protein level. These genotype-specific therapies are now available for an increasing number of CF patients, transforming the way we view the condition from a life-limiting disease to one that can be effectively managed. Several studies have demonstrated the vast improvement CFTR modulators have on normalization of sweat chloride, CFTR function, clinical endpoints, and frequency of pulmonary exacerbation. However, their impact on other aspects of the disease, such as pathogenic burden and airway infection, remain under explored. Frequent airway infections as a result of increased susceptibility and impaired innate immune response are a serious problem within CF, often leading to accelerated decline in lung function and disease progression. Current evidence suggests that CFTR modulators are unable to eradicate pathogenic organisms in those with already established lung disease. However, this may not be the case for those with relatively low levels of disease progression and conserved microbial diversity, such as young patients. Furthermore, it remains unknown whether the restorative effects exerted by CFTR modulators extend to immune cells, such as phagocytes, which have the potential to modulate the response of people with CF (pwCF) to infection. Throughout this review, we look at the potential impact of CFTR modulators on airway infection in CF and their ability to shape impaired pulmonary defences to pathogens. Full article
(This article belongs to the Special Issue Immune Response in Cystic Fibrosis: Interplay between the Host and Microbes)
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