title: A synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells.
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.
authors.affiliation: Department of Physiology, The Chinese University of Hong Kong, Shatin, Hong Kong, China.
authors.forename: Bing
authors.initials: B
authors.lastname: Shen
authors.forename: Xiang
authors.initials: X
authors.lastname: Li
authors.forename: Fei
authors.initials: F
authors.lastname: Wang
authors.forename: Xiaoqiang
authors.initials: X
authors.lastname: Yao
authors.forename: Dan
authors.initials: D
authors.lastname: Yang
mesh_terms: D017878:4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid / Q000737:chemistry; D012856:4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid / Q000737:chemistry; D000818:Animals; D002460:Cell Line; D018118:Chloride Channels / Q000138:chemical synthesis*; D002712:Chlorides / Q000378:metabolism*; D003550:Cystic Fibrosis / Q000378:metabolism*; D004594:Electrophysiology; D004847:Epithelial Cells / Q000187:drug effects / Q000378:metabolism*; D006801:Humans; D008070:Lipopolysaccharides / Q000494:pharmacology; D051379:Mice; D009544:Niflumic Acid / Q000737:chemistry; D014409:Tumor Necrosis Factor-alpha / Q000097:blood; D062367:ortho-Aminobenzoates / Q000737:chemistry
chemical_list: D018118:Chloride Channels; D002712:Chlorides; D008070:Lipopolysaccharides; D014409:Tumor Necrosis Factor-alpha; D062367:ortho-Aminobenzoates; D012856:4-Acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic Acid; D009544:Niflumic Acid; C001209:fenamic acid; D017878:4,4'-Diisothiocyanostilbene-2,2'-Disulfonic Acid