Control of the Proinflammatory State in Cystic Fibrosis Lung Epithelial Cells by Genes from the TNF-αR/NFκB Pathway

Abstract excerpt

BACKGROUND: Cystic fibrosis (CF) is the most common, lethal autosomal recessive disease affecting children in the United States and Europe. Extensive work is being performed to develop both gene and drug therapies. The principal mutation causing CF is in the CFTR gene ([Delta F508]CFTR). This mutation causes the mutant protein to traffic poorly to the plasma membrane, and degrades CFTR chloride channel activity....

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