Cystic fibrosis lung disease starts in the small airways: can we treat it more effectively?

HAWM Tiddens, SH Donaldson… - Pediatric …, 2010 - Wiley Online Library
The aims of this article are to summarize existing knowledge regarding the pathophysiology
of small airways disease in cystic fibrosis (CF), to speculate about additional mechanisms …

Regulation of the epithelial Na+ channel and airway surface liquid volume by serine proteases

EA Gaillard, P Kota, M Gentzsch, NV Dokholyan… - … -European Journal of …, 2010 - Springer
Mammalian airways are protected from infection by a thin film of airway surface liquid (ASL)
which covers airway epithelial surfaces and acts as a lubricant to keep mucus from adhering …

Airway wall stiffening increases peak wall shear stress: a fluid–structure interaction study in rigid and compliant airways

G Xia, MH Tawhai, EA Hoffman, CL Lin - Annals of biomedical engineering, 2010 - Springer
The airflow characteristics in a computed tomography (CT) based human airway bifurcation
model with rigid and compliant walls are investigated numerically. An in-house three …

Cystic fibrosis transmembrane conductance regulator is involved in airway epithelial wound repair

KR Schiller, PJ Maniak… - American Journal of …, 2010 - journals.physiology.org
The role of cystic fibrosis (CF) transmembrane conductance regulator (CFTR) in airway
epithelial wound repair was investigated using normal human bronchial epithelial (NHBE) …

[HTML][HTML] Human alveolar type II cells secrete and absorb liquid in response to local nucleotide signaling

PF Bove, BR Grubb, SF Okada, CMP Ribeiro… - Journal of Biological …, 2010 - ASBMB
A balance sheet describing the integrated homeostasis of secretion, absorption, and surface
movement of liquids on pulmonary surfaces has remained elusive. It remains unclear …

Acute regulation of the epithelial sodium channel in airway epithelia by proteases and trafficking

MM Myerburg, PR Harvey, EM Heidrich… - American journal of …, 2010 - atsjournals.org
Effective clearance of inhaled pathogens is the primary innate defense mechanism in the
lung, and requires the maintenance of a proper airway surface liquid (ASL) volume to …

Recent advances and new opportunities in lung mechanobiology

DJ Tschumperlin, F Boudreault, F Liu - Journal of biomechanics, 2010 - Elsevier
Lung function is inextricably linked to mechanics. On short timescales every breath
generates dynamic cycles of cell and matrix stretch, along with convection of fluids in the …

[HTML][HTML] Airway surface liquid volume regulation determines different airway phenotypes in liddle compared with βENaC-overexpressing mice

MA Mall, B Button, B Johannesson, Z Zhou… - Journal of biological …, 2010 - ASBMB
Studies in cystic fibrosis patients and mice overexpressing the epithelial Na+ channel β-
subunit (βENaC-Tg) suggest that raised airway Na+ transport and airway surface liquid …

Receptor‐promoted exocytosis of airway epithelial mucin granules containing a spectrum of adenine nucleotides

SM Kreda, L Seminario‐Vidal… - The Journal of …, 2010 - Wiley Online Library
Purinergic regulation of airway innate defence activities is in part achieved by the release of
nucleotides from epithelial cells. However, the mechanisms of airway epithelial nucleotide …

Lessons learned from a randomized trial of airway secretion clearance techniques in cystic fibrosis

MK Sontag, AL Quittner, AC Modi… - Pediatric …, 2010 - Wiley Online Library
Rationale Airway secretion clearance therapies are a cornerstone of cystic fibrosis care,
however longitudinal comparative studies are rare. Our objectives were to compare three …