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Cystic fibrosis lung disease: from basic research to clinical issues

Submission deadline: 31 December 2021
Special Issue Editors
Massimo Conese, MD
University of Foggia, Foggia, Italy
Interests: CFTR interactome; innate immunity and inflammation; cell-based therapies; biomarkers
Special Issue and Collections in IMR journals
Special Issue in Cystic fibrosis
Special Issue in Cystic fibrosis
Lorenzo Guerra, MD
University of Bari “A. Moro”, Bari, Italy
Interests: Regulation of CFTR by protein-protein interactions; expression and structure/function of the transporters in intracellular pH regulation; signal transduction
Special Issue and Collections in IMR journals
Special Issue in Cystic fibrosis
Special Issue in Cystic fibrosis
Special Issue Information

Dear Colleadgues,

Cystic fibrosis (CF) is the most common autosomal recessive diseases found within the Caucasian population, whose median survival has been increased due the introduction of novel therapeutic approaches. Although it is a syndrome, the main morbidity and mortality of CF people is caused by the lung disease. The lung involvement is usually progressive with intermittent exacerbations. CF is caused by mutations in the CF Transmembrane Conductance Regulator (CFTR) gene, a chloride channel, involved in the regulation of ion and fluid transport across different epithelium-lined organs. The genetics of CF is quite complex, with genotype-phenotype correlations depending on other genetic and environmental modifiers. The lack/defect of the CFTR protein leads to a dehydrated mucus, impaired mucus clearance, determining the formation of endobronchial mucus plaques and plugs, which become the main sites of air flow obstruction, infection, and inflammation. The immune system, both innate and adaptive, seems to be primarily deranged, contributing to this pathophysiological process. The CFTR protein has been also involved in as many as different processes, such as foetal development, epithelial differentiation/polarization, and regeneration, as well as in epithelial–mesenchymal transition occurring during wound healing and cancer. In this issue, basic research and translational issues will be considered, including genetics of CFTR and other ion channel/transporters, aspects related to inflammation and immunity, as well as tumorigenesis. The identification of novel biomarkers of the lung disease that are specific, can identify CF children at risk for more progressive lung disease and serve as outcome measures for clinical trials is another intensive field of investigation. Finally, current therapeutic modalities, novel pharmacological and genetic therapies targeting the basic defect, cell-based therapies, and lung transplantation will be covered.

Prof. Massimo Coneseand Prof. Lorenzo Guerra

Guest Editors

Keywords
Cystic Fibrosis
Lung Disease
Inflammation
Diagnosis
Therapies
Manuscript Submission Information

Manuscripts should be submitted via our online editorial system at https://imr.propub.com by registering and logging in to this website. Once you are registered, click here to start your submission. Manuscripts can be submitted now or up until the deadline. All papers will go through peer-review process. Accepted papers will be published in the journal (as soon as accepted) and meanwhile listed together on the special issue website. Research articles, reviews as well as short communications are preferred. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office to announce on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts will be thoroughly refereed through a double-blind peer-review process. Please visit the Instruction for Authors page before submitting a manuscript. The Article Processing Charge (APC) in this open access journal is 2500 USD. Submitted manuscripts should be well formatted in good English.

Published Paper (3 Papers)
Open Access Original Research
Characterization of the Extracellular Volatile Metabolome of Pseudomonas Aeruginosa Applying an in vitro Biofilm Model under Cystic Fibrosis-Like Conditions
Timo Koehler, Jost Wingender, Michelle Lueling, Sven W. Meckelmann, ... Oliver J. Schmitz
Front. Biosci. (Landmark Ed) 2022, 27(5), 156; https://doi.org/10.31083/j.fbl2705156
(This article belongs to the Special Issue Cystic fibrosis lung disease: from basic research to clinical issues)
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Open Access Short Communication
Unconventional T Cell Immunity in the Lungs of Young Children with Cystic Fibrosis
Rebecca McElroy, Ghazal Alipour Talesh, Christopher M. Harpur, Rosemary Carzino, ... Philip Sutton
Front. Biosci. (Landmark Ed) 2022, 27(5), 149; https://doi.org/10.31083/j.fbl2705149
(This article belongs to the Special Issue Cystic fibrosis lung disease: from basic research to clinical issues)
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Open Access Review
What is the role of Achromobacter species in patients with cystic fibrosis?
Susanna Esposito, Giovanna Pisi, Valentina Fainardi, Nicola Principi
Front. Biosci. (Landmark Ed) 2021, 26(12), 1613–1620; https://doi.org/10.52586/5054
(This article belongs to the Special Issue Cystic fibrosis lung disease: from basic research to clinical issues)
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