Overview of CF lung pathophysiology

G Cabrini, A Rimessi, M Borgatti, P Pinton… - Current Opinion in …, 2022 - Elsevier
Defects of the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) protein
affect the homeostasis of chloride, bicarbonate, sodium, and water in the airway surface …

Pathophysiology of cystic fibrosis

SH Donaldson, RC Boucher - Annales Nestlé (English ed.), 2006 - karger.com
Understanding the development of lung disease in cystic fibrosis (CF) has been a sought
after goal for several decades. With the cloning of the CF transmembrane conductance …

[HTML][HTML] CFTR modulator therapies: potential impact on airway infections in cystic fibrosis

F Saluzzo, L Riberi, B Messore, NI Loré, I Esposito… - Cells, 2022 - mdpi.com
Cystic Fibrosis (CF) is an autosomal recessive disease caused by mutations in the gene
encoding for the Cystic Fibrosis Transmembrane conductance Regulator (CFTR) protein …

[HTML][HTML] New developments in cystic fibrosis airway inflammation

N Palaniyar, MA Mall, C Taube, S Worgall… - Mediators of …, 2015 - ncbi.nlm.nih.gov
Cystic fibrosis (CF) is an autosomal recessive disease that is caused by mutations in the
cystic fibrosis conductance regulator (CFTR) gene and usually presents with multiorgan …

[HTML][HTML] Dysfunctional inflammation in cystic fibrosis airways: from mechanisms to novel therapeutic approaches

A Ghigo, G Prono, E Riccardi, V De Rose - International Journal of …, 2021 - mdpi.com
Cystic fibrosis (CF) is an inherited disorder caused by mutations in the gene encoding for the
cystic fibrosis transmembrane conductance regulator (CFTR) protein, an ATP-gated chloride …

Cystic fibrosis: pathophysiology of lung disease

C Bergeron, AM Cantin - Seminars in respiratory and critical …, 2019 - thieme-connect.com
Cystic fibrosis (CF) is a common, life-threatening, multisystemic, autosomal recessive
disorder. In the last few years, giant steps have been made with regard to the understanding …

[HTML][HTML] CFTR is a modulator of airway inflammation

BK Rubin - American Journal of Physiology-Lung Cellular …, 2007 - journals.physiology.org
ALTHOUGH IT HAS BEEN 17 YEARS since the identification of the cystic fibrosis
transmembrane ion receptor (CFTR) gene and protein, it remains enigmatic how …

[HTML][HTML] The yin and yang of cystic fibrosis transmembrane conductance regulator function: implications for chronic lung disease

N Vij, GP Downey - American journal of respiratory and critical care …, 2013 - atsjournals.org
The cystic fibrosis (CF) transmembrane conductance regulator (CFTR) is a cAMP-activated
anion channel, primarily expressed in ciliated epithelial cells that have an endoluminal …

Pathophysiology of cystic fibrosis lung disease

MA Mall, RC Boucher - Cystic fibrosis. European Respiratory …, 2014 - books.google.com
Chronic obstructive lung disease starting in the first months of life remains the major cause
of morbidity and mortality in patients with cystic fibrosis (CF). The discovery of the cystic …

Cystic fibrosis transmembrane conductance regulator in COPD: a role in respiratory epithelium and beyond

MA Mall, GJ Criner, M Miravitlles… - European …, 2023 - Eur Respiratory Soc
The cystic fibrosis transmembrane conductance regulator (CFTR) is a crucial ion channel for
transport of chloride and bicarbonate anions. Functional roles of CFTR have been identified …