作者
Robert Tarran, Brian Button, Maryse Picher, Anthony M Paradiso, Carla M Ribeiro, Eduardo R Lazarowski, Liqun Zhang, Peter L Collins, Raymond J Pickles, Jeffrey J Fredberg, Richard C Boucher
发表日期
2005/10/21
期刊
Journal of Biological Chemistry
卷号
280
期号
42
页码范围
35751-35759
出版商
Elsevier
简介
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 (static) culture conditions revealed that normal airway epithelia possess an adenosine-regulated pathway that blends Na+ absorption and Cl- secretion to optimize PCL volume. In cystic fibrosis (CF), the absence of CF transmembrane conductance regulator results in a failure of adenosine regulation of PCL volume, which is predicted to initiate mucus stasis and infection. However, under conditions that mimic the phasic motion of the lung in vivo, ATP release into PCL was increased, CF ion transport was rebalanced, and PCL volume was restored to levels adequate for lung defense. This ATP signaling system was vulnerable, however, to insults that trigger CF bacterial infections, such as viral (respiratory syncitial virus) infections …
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