CFTR and lung homeostasis.
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.
Department of Cell, Developmental and Integrative Biology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama; Pulmonary Injury and Repair Center, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama; and Gregory Fleming James Cystic Fibrosis Center, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama jcollawn@uab.edu.
James F
JF
Collawn
Department of Anesthesiology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama; Department of Cell, Developmental and Integrative Biology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama; Pulmonary Injury and Repair Center, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama; and Gregory Fleming James Cystic Fibrosis Center, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabama.
Sadis
S
Matalon
D000818:Animals; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D006706:Homeostasis; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008168:Lung / Q000378:metabolism* / Q000473:pathology; D029424:Pulmonary Disease, Chronic Obstructive / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D012131:Respiratory Insufficiency / Q000235:genetics / Q000378:metabolism* / Q000473:pathology
C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
chronic obstructive pulmonary disease; cystic fibrosis; inflammatory responses; mucus obstruction; oxidative stress