[HTML][HTML] Evidence for periciliary liquid layer depletion, not abnormal ion composition, in the pathogenesis of cystic fibrosis airways disease
H Matsui, BR Grubb, R Tarran, SH Randell, JT Gatzy… - Cell, 1998 - cell.com
The pathogenesis of cystic fibrosis (CF) airways infection is unknown. Two hypotheses,"
hypotonic [low salt]/defensin" and" isotonic volume transport/mucus clearance," attempt to …
hypotonic [low salt]/defensin" and" isotonic volume transport/mucus clearance," attempt to …
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) …
An overview of the pathogenesis of cystic fibrosis lung disease
RC Boucher - Advanced drug delivery reviews, 2002 - Elsevier
The pathogenesis of cystic fibrosis (CF) lung disease is reviewed, focusing on an overview
of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed …
of the physiologic mechanisms that regulate mucus transport. A major emphasis is placed …
New concepts of the pathogenesis of cystic fibrosis lung disease
RC Boucher - European Respiratory Journal, 2004 - Eur Respiratory Soc
Although there has been impressive progress in the elucidation of the genetic and molecular
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …
basis of cystic fibrosis (CF), the pathogenesis of CF lung disease remains obscure. The …
Airway surface liquid homeostasis in cystic fibrosis: pathophysiology and therapeutic targets
Cystic fibrosis (CF) is a life-limiting disease characterised by recurrent respiratory infections,
inflammation and lung damage. The volume and composition of the airway surface liquid …
inflammation and lung damage. The volume and composition of the airway surface liquid …
Regulation of the depth and composition of airway surface liquid
JH Widdicombe - Journal of anatomy, 2002 - Wiley Online Library
The airways are lined with a film of liquid about 10 µm deep that is in two layers. Around the
cilia is the watery periciliary sol. Over this is a mucous blanket that traps inhaled particles …
cilia is the watery periciliary sol. Over this is a mucous blanket that traps inhaled particles …
[HTML][HTML] Normal and cystic fibrosis airway surface liquid homeostasis: the effects of phasic shear stress and viral infections
Mammalian airways normally regulate the volume of a thin liquid layer, the periciliary liquid
(PCL), to facilitate the mucus clearance component of lung defense. Studies under standard …
(PCL), to facilitate the mucus clearance component of lung defense. Studies under standard …
Role of airway surface liquid and submucosal glands in cystic fibrosis lung disease
AS Verkman, Y Song… - American Journal of …, 2003 - journals.physiology.org
Cystic fibrosis (CF) is caused by mutations in the CF transmembrane conductance regulator
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …
(CFTR) protein, an epithelial chloride channel expressed in the airways, pancreas, testis …
Cystic fibrosis: a disease of vulnerability to airway surface dehydration
RC Boucher - Trends in molecular medicine, 2007 - cell.com
Cystic fibrosis (CF) lung disease involves chronic bacterial infection of retained airway
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …
secretions (mucus). Recent data suggest that CF lung disease pathogenesis reflects the …
[HTML][HTML] The CF salt controversy: in vivo observations and therapeutic approaches
There is controversy over whether abnormalities in the salt concentration or volume of
airway surface liquid (ASL) initiate cystic fibrosis (CF) airway disease. In vivo studies of CF …
airway surface liquid (ASL) initiate cystic fibrosis (CF) airway disease. In vivo studies of CF …