Small-molecule ion channels increase host defences in cystic fibrosis airway epithelia.

ABSTRACT: Loss-of-function mutations in the cystic fibrosis transmembrane conductance regulator (CFTR) compromise epithelial HCO₃^- and Cl^- secretion, reduce airway surface liquid pH, and impair respiratory host defences in people with cystic fibrosis^1-3. Here we report that apical addition of amphotericin B, a small molecule that forms unselective ion channels, restored HCO₃^- secretion and increased airway surface liquid pH in cultured airway epithelia from people with cystic fibrosis. These effects required the basolateral Na⁺, K⁺-ATPase, indicating that apical amphotericin B channels functionally interfaced with this driver of anion secretion. Amphotericin B also restored airway surface liquid pH, viscosity, an

SUBMITTER: Muraglia KA 

PROVIDER: S-EPMC6492938 | biostudies-literature | 2019 Mar

REPOSITORIES: biostudies-literature