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Review
. 2014 Dec 15;307(12):L917-23.
doi: 10.1152/ajplung.00326.2014. Epub 2014 Nov 7.

CFTR and lung homeostasis

James F Collawn  1 , Sadis Matalon  2
Affiliations
Review

CFTR and lung homeostasis

James F Collawn et al. Am J Physiol Lung Cell Mol Physiol. .

Abstract

CFTR is a cAMP-activated chloride and bicarbonate channel that is critical for lung homeostasis. Decreases in CFTR expression have dire consequences in cystic fibrosis (CF) and have been suggested to be a component of the lung pathology in chronic obstructive pulmonary disease. Decreases or loss of channel function often lead to mucus stasis, chronic bacterial infections, and the accompanying chronic inflammatory responses that promote progressive lung destruction, and, eventually in CF, lung failure. Here we discuss CFTR's functional role airway surface liquid hydration and pH, in regulation of other channels such as the epithelial sodium channel, and in regulating inflammatory responses in the lung.

Keywords: chronic obstructive pulmonary disease; cystic fibrosis; inflammatory responses; mucus obstruction; oxidative stress.

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