Liquid movement across the surface epithelium of large airways

LA Chambers, BM Rollins, R Tarran - Respiratory physiology & …, 2007 - Elsevier
The cystic fibrosis transmembrane conductance regulator CFTR gene is found on
chromosome 7 [Kerem, B., Rommens, JM, Buchanan, JA, Markiewicz, D., Cox, TK …

The epithelial sodium channel (ENaC) as a therapeutic target for cystic fibrosis lung disease

PJ Moore, R Tarran - Expert opinion on therapeutic targets, 2018 - Taylor & Francis
Introduction: Cystic fibrosis is an autosomal recessive disorder caused by mutations in the
cystic fibrosis transmembrane conductance regulator (CFTR) gene that codes for the CFTR …

Junctional abnormalities in human airway epithelial cells expressing F508del CFTR

SA Molina, B Stauffer, HK Moriarty… - … of Physiology-Lung …, 2015 - journals.physiology.org
Cystic fibrosis (CF) has a profound impact on airway physiology. Accumulating evidence
suggests that intercellular junctions are impaired in CF. We examined changes to CF …

Control of epithelial Na+ conductance by the cystic fibrosis transmembrane conductance regulator

K Kunzelmann, R Schreiber, R Nitschke, M Mall - Pflügers Archiv, 2000 - Springer
Cystic fibrosis transmembrane conductance regulator (CFTR) is an epithelial Cl–channel
expressed in luminal membranes of secretory and reabsorptive epithelia. CFTR plays a …

Liquid secretion properties of airway submucosal glands

ST Ballard, SK Inglis - The Journal of physiology, 2004 - Wiley Online Library
The tracheobronchial submucosal glands secrete liquid that is important for hydrating airway
surfaces, supporting mucociliary transport, and serving as a fluid matrix for numerous …

Abnormal respiratory epithelial ion transport in cystic fibrosis

MR Knowles, MJ Stutts, JR Yankaskas, JT Gatzy… - Clinics in chest …, 1986 - Elsevier
Abnormal airway secretions characterize asthma" and cystic fibrosis (CF), 13.73 obstructive
airway diseases with different clinical courses. Whereas airway secretions that are retained …

The role of bacterial secretion systems in the virulence of gram-negative airway pathogens associated with cystic fibrosis

S Depluverez, S Devos, B Devreese - Frontiers in microbiology, 2016 - frontiersin.org
Cystic fibrosis (CF) is the most common lethal inherited disorder in Caucasians. It is caused
by mutation of the CF transmembrane conductance regulator (CFTR) gene. A defect in the …

Cystic fibrosis transmembrane conductance regulator–associated ATP release is controlled by a chloride sensor

Q Jiang, D Mak, S Devidas, EM Schwiebert… - The Journal of cell …, 1998 - rupress.org
The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel that
is defective in cystic fibrosis, and has also been closely associated with ATP permeability in …

Cystic fibrosis transmembrane conductance regulator trafficking modulates the barrier function of airway epithelial cell monolayers

P LeSimple, J Liao, R Robert… - The Journal of …, 2010 - Wiley Online Library
The cystic fibrosis transmembrane conductance regulator (CFTR) is an integral membrane
glycoprotein which functions as an anion channel and influences diverse cellular processes …

Evidence of a functional CFTR Cl channel in adult alveolar epithelial cells

E Brochiero, A Dagenais, A Privé… - … of Physiology-Lung …, 2004 - journals.physiology.org
The cystic fibrosis transmembrane conductance regulator (CFTR) is expressed in the fetal
lung, but during lung development it gradually disappears in cells of future alveolar spaces …