Progress in cystic fibrosis and the CF Therapeutics Development Network
SM Rowe, DS Borowitz, JL Burns, JP Clancy… - Thorax, 2012 - thorax.bmj.com
Cystic fibrosis (CF), the most common life-shortening genetic disorder in Caucasians, affects
approximately 70 000 individuals worldwide. In 1998, the Cystic Fibrosis Foundation (CFF) …
approximately 70 000 individuals worldwide. In 1998, the Cystic Fibrosis Foundation (CFF) …
Lung inflammation in cystic fibrosis: pathogenesis and novel therapies
Despite remarkable progress following the identification of the causing gene, the final
outcome of cystic fibrosis (CF) remains determined mainly by the progressive reduction of …
outcome of cystic fibrosis (CF) remains determined mainly by the progressive reduction of …
[HTML][HTML] Recent progress in translational cystic fibrosis research using precision medicine strategies
DM Cholon, M Gentzsch - Journal of Cystic Fibrosis, 2018 - Elsevier
Significant progress has been achieved in developing precision therapies for cystic fibrosis;
however, highly effective treatments that target the ion channel, CFTR, are not yet available …
however, highly effective treatments that target the ion channel, CFTR, are not yet available …
Coupling of airway ciliary activity and mucin secretion to mechanical stresses by purinergic signaling
CW Davis, E Lazarowski - Respiratory physiology & neurobiology, 2008 - Elsevier
The mucociliary clearance system is comprised of three components, ion transport activities
controlling the height of airway surface liquid (ASL), mucin secretion, and ciliary activity …
controlling the height of airway surface liquid (ASL), mucin secretion, and ciliary activity …
Inhaled hypertonic saline as a therapy for cystic fibrosis
MR Elkins, PTP Bye - Current opinion in pulmonary medicine, 2006 - journals.lww.com
Inhaled hypertonic saline as a therapy for cystic fibrosis : Current Opinion in Pulmonary
Medicine Inhaled hypertonic saline as a therapy for cystic fibrosis : Current Opinion in …
Medicine Inhaled hypertonic saline as a therapy for cystic fibrosis : Current Opinion in …
Mechano-sensitivity of ENaC: may the (shear) force be with you
M Fronius, WG Clauss - Pflügers Archiv-European Journal of Physiology, 2008 - Springer
Abstract The epithelial Na+ channel (ENaC) is the rate-limiting step for Na+ absorption in
various vertebrate epithelia and deeply enmeshed in the control of salt and water …
various vertebrate epithelia and deeply enmeshed in the control of salt and water …
Spatiotemporal organization of cilia drives multiscale mucus swirls in model human bronchial epithelium
MK Khelloufi, E Loiseau, M Jaeger, N Molinari… - Scientific reports, 2018 - nature.com
Mucociliary clearance is a biomechanical mechanism of airway protection. It consists of the
active transport along the bronchial tree of the mucus, a fluid propelled by the coordinated …
active transport along the bronchial tree of the mucus, a fluid propelled by the coordinated …
Particle transport and deposition correlation with near-wall flow characteristic under inspiratory airflow in lung airways
Exposure of lung airways to detrimental suspended aerosols in the environment increases
the vulnerability of the respiratory and cardiovascular systems. In addition, recent …
the vulnerability of the respiratory and cardiovascular systems. In addition, recent …
Mass spectrometric analysis of biomarkers and dilution markers in exhaled breath condensate reveals elevated purines in asthma and cystic fibrosis
CR Esther Jr, G Boysen, BM Olsen… - … of Physiology-Lung …, 2009 - journals.physiology.org
Exhaled breath condensate (EBC) analyses promise simple and noninvasive methods to
measure airway biomarkers but pose considerable methodological challenges. We utilized …
measure airway biomarkers but pose considerable methodological challenges. We utilized …
Regulation of cation transport in the distal nephron by mechanical forces
LM Satlin, MD Carattino, W Liu… - American Journal of …, 2006 - journals.physiology.org
Thiazide and loop diuretics induce renal K+ secretion, often leading to renal K+ wasting and
hypokalemia. This phenomenon has been proposed to reflect an increase in delivery to and …
hypokalemia. This phenomenon has been proposed to reflect an increase in delivery to and …