Ivacaftor restores delayed mucociliary transport caused by Pseudomonas aeruginosa-induced acquired cystic fibrosis transmembrane conductance regulator dysfunction in rabbit nasal epithelia.
Do-Yeon ChoShaoyan ZhangDaniel F SkinnerDong Jin LimCatherine G BanksJessica W GraysonGuillermo J TearneySteven M RoweBradford A WoodworthPublished in: International forum of allergy & rhinology (2021)
In rabbit nasal epithelium, ivacaftor robustly stimulates CFTR-mediated Cl- secretion and normalizes ASL and CBF in PAO1-induced acquired CFTR dysfunction. Preclinical testing of CFTR potentiators as therapy for P aeruginosa rabbit sinusitis is planned.