Epithelial Cell Line Expressing a Cystic Fibrosis Phenotype

Description:

Cystic fibrosis (CF) is a common genetic disease that affects the entire body, producing thick, sticky mucus that clogs the lungs, pancreas, and other organs. It is the most common fatal genetic disease in the United States, and is caused by a mutation in the cystic fibrosis transmembrane conductance regulator (CFTR).

Researchers at NIEHS have developed a cell line, CF/T43, which was produced by infection of airway epithelial cells isolated from CF patients with an SV40T retrovirus. CF/T43 cells maintain the abnormal ion transport characteristics of CF while having proliferation capability beyond that of a primary epithelial cell culture. Key features of the CF/T43 cell line include the formation of functional tight junctions, reduced apical membrane chloride conductance, and activation of apical chloride channels by calcium ionophores but not by cAMP-dependent agonists. This cell line may be used for elucidation of the mechanisms of CF, testing candidate complementary genes for correction of the observed CF abnormalities, and for developing and testing therapeutic CF drugs.