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Overview of CF lung pathophysiology |
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| Institution: | 1. Department of Physiology, McGill University, Montréal, Canada;2. Department of Pharmaceutical and Pharmacological Sciences, University of Padova, Padova, Italy.;3. Department of Biochemistry, McGill University, Montréal, Canada. |
| Abstract: | Defects of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface liquid, influencing the mucus composition and viscosity, which induces a severe condition of infection and inflammation along the whole life of CF patients. The introduction of CFTR modulators, novel drugs directly intervening to rescue the function of CFTR protein, opens a new era of experimental research. The review summarizes the most recent advancements to understand the characteristics of the infective and inflammatory pathology of CF lungs. |
| Keywords: | Cystic fibrosis CFTR modulators Lung inflammation Reactive oxygen species CF"} {"#name":"keyword" "$":{"id":"pc_QLQbUfGuaw"} "$$":[{"#name":"text" "_":"Cystic Fibrosis CFTR"} {"#name":"keyword" "$":{"id":"pc_28msXNzhqQ"} "$$":[{"#name":"text" "_":"Cystic Fibrosis Transmembrane conductance Regulator IL-8"} {"#name":"keyword" "$":{"id":"pc_L74UMz4P0T"} "$$":[{"#name":"text" "_":"Interleukin 8 ENaC"} {"#name":"keyword" "$":{"id":"pc_QRnRDBom4D"} "$$":[{"#name":"text" "_":"Epithelium Sodium Channel ASL"} {"#name":"keyword" "$":{"id":"pc_LFx2vQQPqi"} "$$":[{"#name":"text" "_":"Airway Surface Liquid BALF"} {"#name":"keyword" "$":{"id":"pc_0GIciMc0D5"} "$$":[{"#name":"text" "_":"Bronchoalveolar Lavage Fluid PMN"} {"#name":"keyword" "$":{"id":"pc_6OcvK8hKzP"} "$$":[{"#name":"text" "_":"PolyMorphonuclear Neutrophil PTEN"} {"#name":"keyword" "$":{"id":"pc_wQwJ7hRFCF"} "$$":[{"#name":"text" "_":"phosphatase and tensin homolog deleted in chromosome 10 |
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