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 …

Increased airway epithelial Na+ absorption produces cystic fibrosis-like lung disease in mice

M Mall, BR Grubb, JR Harkema, WK O'Neal… - Nature medicine, 2004 - nature.com
Mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene result in
defective epithelial cAMP-dependent Cl− secretion and increased airway Na+ absorption …

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 …

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 …

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 …

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 …

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 …

Pseudomonas aeruginosa inhibits endocytic recycling of CFTR in polarized human airway epithelial cells

A Swiatecka-Urban… - … of physiology-cell …, 2006 - journals.physiology.org
The most common mutation in the CFTR gene in individuals with cystic fibrosis (CF), ΔF508,
leads to the absence of CFTR Cl− channels in the apical plasma membrane, which in turn …