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) …

Lung inflammation in cystic fibrosis: pathogenesis and novel therapies

B Dhooghe, S Noël, F Huaux, T Leal - Clinical biochemistry, 2014 - Elsevier
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 …

[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 …

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 …

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 …

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 …

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 …

Particle transport and deposition correlation with near-wall flow characteristic under inspiratory airflow in lung airways

A Farghadan, K Poorbahrami, S Jalal, JM Oakes… - Computers in biology …, 2020 - Elsevier
Exposure of lung airways to detrimental suspended aerosols in the environment increases
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 …

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 …