Electrolyte transport properties in distal small airways from cystic fibrosis pigs with implications for host defense

American Journal of Physiology. Lung Cellular and Molecular Physiology
Xiaopeng LiJoseph Zabner

Abstract

While pathological and clinical data suggest that small airways are involved in early cystic fibrosis (CF) lung disease development, little is known about how the lack of cystic fibrosis transmembrane conductance regulator (CFTR) function contributes to disease pathogenesis in these small airways. Large and small airway epithelia are exposed to different airflow velocities, temperatures, humidity, and CO2 concentrations. The cellular composition of these two regions is different, and small airways lack submucosal glands. To better understand the ion transport properties and impacts of lack of CFTR function on host defense function in small airways, we adapted a novel protocol to isolate small airway epithelial cells from CF and non-CF pigs and established an organotypic culture model. Compared with non-CF large airways, non-CF small airway epithelia cultures had higher Cl(-) and bicarbonate (HCO3 (-)) short-circuit currents and higher airway surface liquid (ASL) pH under 5% CO2 conditions. CF small airway epithelia were characterized by minimal Cl(-) and HCO3 (-) transport and decreased ASL pH, and had impaired bacterial killing compared with non-CF small airways. In addition, CF small airway epithelia had a higher ASL viscosit...Continue Reading

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Citations

Aug 4, 2016·American Journal of Respiratory Cell and Molecular Biology·Luis G Vargas BuonfiglioAlejandro P Comellas
Jan 27, 2017·American Journal of Physiology. Cell Physiology·Bruce A Stanton
Mar 9, 2018·Journal of Applied Physiology·Yunxia O'MalleyAliye Uc
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Feb 4, 2021·The Journal of Experimental Medicine·Dusik KimJohn W Hanrahan
Apr 1, 2021·American Journal of Respiratory Cell and Molecular Biology·Xiaopeng LiJoseph Zabner
Nov 25, 2021·Science Translational Medicine·Joan K LunneyChaohui Dai

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