The CFTR and ENaC debate: how important is ENaC in CF lung disease?

• Published in: American journal of physiology. Lung cellular and molecular physiology Vol. 302; no. 11; pp. L1141 - L1146
• Main Authors: Collawn, James F., Lazrak, Ahmed, Bebok, Zsuzsa, Matalon, Sadis
• Format: Journal Article
• Language: English
• Published: United States American Physiological Society 01.06.2012
• Subjects: Animals; Bacteria; Bacteria - immunology; Bacteria - pathogenicity; Bacterial diseases; Bacterial Infections - metabolism; Bacterial Infections - pathology; Cystic fibrosis; Cystic Fibrosis - genetics; Cystic Fibrosis - metabolism; Cystic Fibrosis - pathology; Cystic Fibrosis Transmembrane Conductance Regulator - deficiency; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Disease Models, Animal; Epithelial Sodium Channels - genetics; Epithelial Sodium Channels - metabolism; Genotype & phenotype; Humans; Ion Transport; Lung - metabolism; Lung - pathology; Lung diseases; Lung Diseases - genetics; Lung Diseases - metabolism; Lung Diseases - pathology; Mice; Mice, Transgenic; Mortality; Swine
• ISSN: 1040-0605; 1522-1504; 1522-1504
• DOI: 10.1152/ajplung.00036.2012

Cystic fibrosis (CF) is caused by the loss of the cystic fibrosis transmembrane conductance regulator (CFTR) function and results in a respiratory phenotype that is characterized by dehydrated mucus and bacterial infections that affect CF patients throughout their lives. Much of the morbidity and mortality in CF results from a failure to clear bacteria from the lungs. What causes the defect in the bacterial clearance in the CF lung has been the subject of an ongoing debate. Here we discuss the arguments for and against the role of the epithelial sodium channel, ENaC, in the development of CF lung disease.