A Synthetic Chloride Channel Restores Chloride Conductance in Human Cystic Fibrosis Epithelial Cells

by Bing Shen, Xiang Li, Fei Wang, Xiaoqiang Yao, Dan Yang

Mutations in the gene-encoding cystic fibrosis transmembrane conductance regulator (CFTR) cause defective transepithelial transport of chloride (Cl⁻) ions and fluid, thereby becoming responsible for the onset of cystic fibrosis (CF). One strategy to reduce the pathophysiology associated with CF is to increase Cl⁻ transport through alternative pathways. In this paper, we demonstrate that a small synthetic molecule which forms Cl⁻ channels to mediate Cl⁻ transport across lipid bilayer membranes is capable of restoring Cl⁻ permeability in human CF epithelial cells; as a result, it has the potential to become a lead compound for the treatment of human diseases associated with Cl⁻ channel dysfunction.

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Syndicated from:PLoS ONE

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