A synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells |
| |
Authors: | Shen Bing Li Xiang Wang Fei Yao Xiaoqiang Yang Dan |
| |
Affiliation: | Department of Physiology, The Chinese University of Hong Kong, Shatin, Hong Kong, China. |
| |
Abstract: | 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. |
| |
Keywords: | |
本文献已被 PubMed 等数据库收录! |
|