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2014 ; 307
(12
): L917-23
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CFTR and lung homeostasis
#MMPMID25381027
Collawn JF
; Matalon S
Am J Physiol Lung Cell Mol Physiol
2014[Dec]; 307
(12
): L917-23
PMID25381027
show ga
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.