Mechanistic review reveals how regulator mutations disrupt airway-surface liquid and mucociliary transport in cystic fibrosis, highlighting drivers of lung disease.
Mutations in the gene encoding the cystic fibrosis transmembrane conductance regulator undermine many host defense systems by inhibiting the function of airway-surface liquid, causing flaws in mucociliary transport, and compromising other lung-protection mechanisms.
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Stoltz et al. (2015) studied this question.
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