[HTML][HTML] Ion channels as targets to treat cystic fibrosis lung disease
Lung health relies on effective mucociliary clearance and innate immune defence
mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of …
mechanisms. In cystic fibrosis (CF), an imbalance in ion transport due to an absence of …
[HTML][HTML] Could a defective epithelial sodium channel lead to bronchiectasis
I Fajac, M Viel, S Sublemontier, D Hubert… - Respiratory research, 2008 - Springer
Background Bronchiectasis is defined as a permanent dilation of the airways arising from
chronic bronchial inflammation/infection. In 50% of cases, no etiology can be identified …
chronic bronchial inflammation/infection. In 50% of cases, no etiology can be identified …
Wild type but not ΔF508 CFTR inhibits Na+ conductance when coexpressed in Xenopus oocytes
M Mall, A Hipper, R Greger, K Kunzelmann - FEBS letters, 1996 - Wiley Online Library
Airway epithelial cells bearing mutations of the cystic fibrosis (CF) transmembrane
conductance regulator (CFTR) possess an increased Na+ conductance along with their well …
conductance regulator (CFTR) possess an increased Na+ conductance along with their well …
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 …
after goal for several decades. With the cloning of the CF transmembrane conductance …
Rationale for hypertonic saline therapy for cystic fibrosis lung disease
R Tarran, S Donaldson… - Seminars in respiratory …, 2007 - thieme-connect.com
Cystic fibrosis (CF) is caused by alterations in the CF transmembrane conductance regulator
(CFTCR) gene. More than 1400 mutations in the CFTCR gene have been described, but the …
(CFTCR) gene. More than 1400 mutations in the CFTCR gene have been described, but the …
Relative expression of the human epithelial Na+ channel subunits in normal and cystic fibrosis airways
LH Burch, CR Talbot, MR Knowles… - … of Physiology-Cell …, 1995 - journals.physiology.org
The availability of the newly cloned subunits (alpha, beta, gamma) of the epithelial Na+
channel (ENaC) permits molecular studies of the pathogenesis of the abnormal Na+ …
channel (ENaC) permits molecular studies of the pathogenesis of the abnormal Na+ …
Role of CFTR in airway disease
JM Pilewski, RA Frizzell - Physiological reviews, 1999 - journals.physiology.org
Pilewski, Joseph M., and Raymond A. Frizzell. Role of CFTR in Airway Disease. Physiol.
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …
Rev. 79, Suppl.: S215–S255, 1999.—Cystic fibrosis (CF) is caused by mutations in the gene …
Preclinical evaluation of the epithelial sodium channel inhibitor BI 1265162 for treatment of cystic fibrosis
P Nickolaus, B Jung, J Sabater, S Constant… - ERJ Open …, 2020 - Eur Respiratory Soc
Background Epithelial sodium channel (ENaC) is an important regulator of airway surface
liquid volume; ENaC is hyperactivated in cystic fibrosis (CF). ENaC inhibition is a potential …
liquid volume; ENaC is hyperactivated in cystic fibrosis (CF). ENaC inhibition is a potential …
Restoring airway surface liquid in cystic fibrosis
F Ratjen - New England Journal of Medicine, 2006 - Mass Medical Soc
The current pathophysiological model of cystic fibrosis lung disease assumes that defective
expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) …
expression, trafficking, or function of the cystic fibrosis transmembrane regulator (CFTR) …