Article
Epithelial sodium channel silencing as a strategy to correct the airway surface fluid deficit in cystic fibrosis.
American journal of respiratory cell and molecular biology - 1 Sept 2013
Gianotti Ambra, Melani Raffaella, Caci Emanuela, Sondo Elvira, Ravazzolo Roberto, Galietta Luis J V, Zegarra-Moran Olga
Abstract excerpt
In the respiratory system, Na(+) absorption and Cl(-) secretion are balanced to maintain an appropriate airway surface fluid (ASF) volume and ensure efficient mucociliary clearance. In cystic fibrosis (CF), this equilibrium is disrupted by mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) gene, resulting in the absence of functional CFTR-dependent Cl(-) secretion. The consequences of...
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