Molecular Mechanisms and Pathophysiology of Cystic Fibrosis
A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".
Deadline for manuscript submissions: 30 January 2025 | Viewed by 70
Special Issue Editors
Interests: pulmonary medicine; pulmonary physiology and cell biology; aging research
Special Issues, Collections and Topics in MDPI journals
Interests: pulmonary medicine; pulmonary physiology and cell biology; clinical research and trial endpoints; new therapy investigation
Special Issue Information
Dear Colleagues,
Cystic fibrosis (CF) is a multi-system disease caused by variants in the CF transmembrane conductance regulator (CFTR) gene. The resultant protein dysfunction results in a wide variety of pathological mechanisms causing multi system pathology. While CFTR protein modulators improve clinical outcomes in many people with CF (pwCF) and especially reduce aspects of airway pathology, the disease remains progressive, especially in pwCF, who had significant lung function decline before initiation of modulator therapy. Furthermore, many disease manifestations do not respond to CFTR modulator therapy resulting in ongoing morbidity. This special issue welcomes basic and translational research studies that focus on characterizing or exploring pathological processes that may have implications in future treatment and better understanding of disease processes in CF-associated disease manifestations. This issue will emphasize understanding of pathological mechanisms that underpin aging and long-term disease progression, pathological mechanisms in airway and non-airway disease processes, and novel or new understandings of CF pathological mechanisms in the post CFTR modulator era.
Dr. Stefanie Krick
Dr. George M. Solomon
Guest Editors
Manuscript Submission Information
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Keywords
- cystic fibrosis
- aging
- CF airway disease
- CF complications
- CF molecular pathogenesis
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