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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Vydáno v:JCI Insight
Hlavní autoři: 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.
Médium: Artigo
Jazyk:Inglês
Vydáno: American Society for Clinical Investigation 2018
Témata:
Clinical Medicine
On-line přístup: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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