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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.

SUBMITTER: Shen B

PROVIDER: S-EPMC3326041 | biostudies-literature | 2012

REPOSITORIES: biostudies-literature

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A synthetic chloride channel restores chloride conductance in human cystic fibrosis epithelial cells.

Shen Bing B Li Xiang X Wang Fei F Yao Xiaoqiang X Yang Dan D

PloS one 20120413 4

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 memb ...[more]

PMID: 22514656

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