Correlation between Ivacaftor-induced CFTR Activation in Airway Epithelial Cells and Improved Lung Function: A Proof-of-Concept Study

Ann Am Thorac Soc. 2020 Aug;17(8):1024-1027. doi: 10.1513/AnnalsATS.202001-082RL.

Authors

Jason S Debley^{1

2}, Kaitlyn A Barrow¹, Lucille M Rich¹, Pradeep Singh^{2

3}, Edward F McKone^{4

5}, David P Nichols^{1

2}

Affiliations

¹ Seattle Children's Hospital and Research InstituteSeattle, Washington.
² University of Washington School of MedicineSeattle, Washington.
³ University of WashingtonSeattle, Washington.
⁴ St. Vincent University HospitalDublin, Irelandand.
⁵ University College Dublin School of MedicineDublin, Ireland.

No abstract available

Publication types

Letter

MeSH terms

Aminophenols / therapeutic use*
Cystic Fibrosis / drug therapy*
Cystic Fibrosis / physiopathology
Cystic Fibrosis Transmembrane Conductance Regulator / agonists
Epithelial Cells / drug effects*
Humans
Lung / drug effects*
Lung / physiopathology
Proof of Concept Study
Quinolones / therapeutic use*

Substances

Aminophenols
Quinolones
Cystic Fibrosis Transmembrane Conductance Regulator
ivacaftor

Grants and funding