Article
Dynamic regulation of airway surface liquid pH by TMEM16A and SLC26A4 in cystic fibrosis nasal epithelia with rare mutations.
Proceedings of the National Academy of Sciences of the United States of America - 21 Nov 2023
Delpiano Livia, Rodenburg Lisa W, Burke Matthew, Nelson Glyn, Amatngalim Gimano D, Beekman Jeffrey M, Gray Michael A
Abstract excerpt
In cystic fibrosis (CF), defects in the CF transmembrane conductance regulator (CFTR) channel lead to an acidic airway surface liquid (ASL), which compromises innate defence mechanisms, predisposing to pulmonary failure. Restoring ASL pH is a potential therapy for people with CF, particularly for those who cannot benefit from current highly effective modulator therapy. However, we lack a comprehensive...
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