title: The CFTR and ENaC debate: how important is ENaC in CF lung disease?
abstract: 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.
authors.affiliation: Department of Cell, Developmental and Integrative Biology, School of Medicine, University of Alabama at Birmingham, 35294-0005, USA. jcollawn@uab.edu
authors.forename: James F
authors.initials: JF
authors.lastname: Collawn
authors.forename: Ahmed
authors.initials: A
authors.lastname: Lazrak
authors.forename: Zsuzsa
authors.initials: Z
authors.lastname: Bebok
authors.forename: Sadis
authors.initials: S
authors.lastname: Matalon
mesh_terms: D000818:Animals; D001419:Bacteria / Q000276:immunology / Q000472:pathogenicity; D001424:Bacterial Infections / Q000378:metabolism / Q000473:pathology; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism* / Q000473:pathology; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000172:deficiency / Q000235:genetics / Q000378:metabolism*; D004195:Disease Models, Animal; D053503:Epithelial Sodium Channels / Q000235:genetics / Q000378:metabolism*; D006801:Humans; D017136:Ion Transport; D008168:Lung / Q000378:metabolism / Q000473:pathology; D008171:Lung Diseases / Q000235:genetics / Q000378:metabolism / Q000473:pathology; D051379:Mice; D008822:Mice, Transgenic; D013552:Swine
chemical_list: C505032:CFTR protein, human; D053503:Epithelial Sodium Channels; D019005:Cystic Fibrosis Transmembrane Conductance Regulator