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a synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells氯化合成氯通道恢复电导在人类囊性纤维化上皮细胞
A Synthetic Chloride Channel Restores Chloride
Conductance in Human Cystic Fibrosis Epithelial Cells
2,3. 1. 3 2 1
Bing Shen , Xiang Li , Fei Wang , Xiaoqiang Yao *, Dan Yang *
1 Morningside Laboratory for Chemical Biology, Department of Chemistry, The University of Hong Kong, Hong Kong, China, 2 Department of Physiology, The Chinese
University of Hong Kong, Shatin, Hong Kong, China, 3 Department of Physiology, Anhui Medical University, Hefei, China
Abstract
Mutations in the gene-encoding cystic fibrosis transmembrane conductance regulator (CFTR) cause defective transepithelial
transport of chloride (Cl2) 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 Cl2 transport through alternative pathways. In this paper, we
demonstrate that a small synthetic molecule which forms Cl2 channels to mediate Cl2 transport across lipid bilayer
membranes is capable of restoring Cl2 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 Cl2 channel dysfunction.
Citation: Shen B, Li X, Wang F, Yao X, Yang D (2012) A Synthetic Chloride Channel Restores Chloride Conductance in Human Cystic Fibrosis Epithelial Cells. PLoS
ONE 7(4): e34694. doi:10.1371/journal.pone.0034694
Editor: Rafael Linden, Universidade Federal do Rio de Janeiro, Brazil
Received August 18, 2011; Accepted March 5, 2012; Published April 13, 2012
Copyright: 2012 Shen et al. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits
unrestricted use, distribution, and reproduction in any medium, provided the original
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