A Synthetic Chloride Channel Restores Chloride Conductance in Human Cystic Fibrosis Epithelial CellsReport as inadecuate




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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.



Author: Bing Shen , Xiang Li , Fei Wang, Xiaoqiang Yao , Dan Yang

Source: http://plos.srce.hr/



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