Open AccessThe CFTR and ENaC debate: how important is ENaC in CF lung disease?
Author(s) -
James F. Collawn,
Ahmed Lazrak,
Zsuzsa Bebök,
Sadis Matalon
Publication year - 2012
Publication title -
american journal of physiology. lung cellular and molecular physiology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.892
H-Index - 163
eISSN - 1522-1504
pISSN - 1040-0605
DOI - 10.1152/ajplung.00036.2012
Subject(s) - epithelial sodium channel , cystic fibrosis , cystic fibrosis transmembrane conductance regulator , lung , phenotype , lung function , mucus , lung disease , disease , chloride channel , medicine , immunology , biology , microbiology and biotechnology , sodium , chemistry , genetics , gene , ecology , organic chemistry
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.