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

• Published in: The European respiratory journal Vol. 59; no. 2; p. 2100185
• Main Authors: Morrison, Cameron B., Shaffer, Kendall M., Araba, Kenza C., Markovetz, Matthew R., Wykoff, Jason A., Quinney, Nancy L., Hao, Shuyu, Delion, Martial F., Flen, Alexis L., Morton, Lisa C., Liao, Jimmy, Hill, David B., Drumm, Mitchell L., O'Neal, Wanda K., Kesimer, Mehmet, Gentzsch, Martina, Ehre, Camille
• Format: Journal Article
• Language: English
• Published: England 01.02.2022
• Subjects: Bicarbonates - metabolism; Cystic Fibrosis - metabolism; Cystic Fibrosis Transmembrane Conductance Regulator - genetics; Cystic Fibrosis Transmembrane Conductance Regulator - metabolism; Humans; Ion Transport; Mucus - metabolism

Cystic fibrosis (CF) is characterised by the accumulation of viscous adherent mucus in the lungs. While several hypotheses invoke a direct relationship with cystic fibrosis transmembrane conductance regulator (CFTR) dysfunction ( acidic airway surface liquid (ASL) pH, low bicarbonate (HCO ) concentr...