Ivacaftor-induced sweat chloride reductions correlate with increases in airway surface liquid pH in cystic fibrosis

BACKGROUND. Disruption of cystic fibrosis transmembrane conductance regulator (CFTR) anion channel function causes cystic fibrosis (CF), and lung disease produces most of the mortality. Loss of CFTR-mediated HCO(3)(–) secretion reduces the pH of airway surface liquid (ASL) in vitro and in neonatal h...

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Bibliographic Details
Published in:JCI Insight
Main Authors: Alaiwa, Mahmoud H. Abou, Launspach, Jan L., Grogan, Brenda, Carter, Suzanne, Zabner, Joseph, Stoltz, David A., Singh, Pradeep K., McKone, Edward F., Welsh, Michael J.
Format: Artigo
Language:Inglês
Published: American Society for Clinical Investigation 2018
Subjects:
Clinical Medicine
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC6129116/
https://ncbi.nlm.nih.gov/pubmed/30089726
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/jci.insight.121468
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