Treatment of cystic fibrosis airway cells with CFTR modulators reverses aberrant mucus properties via hydration.

Cameron Bradley MorrisonKendall M ShafferKenza C ArabaMatthew R MarkovetzJason A WykoffNancy L QuinneyShuyu HaoMartial F DelionAlexis L FlenLisa C MortonJimmy LiaoDavid B HillMitchell L DrummWanda K O'NealMehmet KesimerMartina GentzschCamille Ehre

Published in: The European respiratory journal (2021)

These results indicate that airway dehydration, not acidic pH and/or low [HCO3 -], is responsible for abnormal mucus properties in CF airways and CFTR modulation predominantly restores normal mucin entanglement.

Keyphrases

cystic fibrosis
pseudomonas aeruginosa
lung function
induced apoptosis
small molecule
ionic liquid
endoplasmic reticulum stress
cell proliferation
combination therapy
air pollution