Article
Activation of NF-kappaB in airway epithelial cells is dependent on CFTR trafficking and Cl- channel function.
American journal of physiology. Lung cellular and molecular physiology - 1 Jul 2001
Weber A J, Soong G, Bryan R, Saba S, Prince A
Abstract excerpt
Polymorphonuclear leukocyte-dominated airway inflammation is a major component of cystic fibrosis (CF) lung disease and may be associated with CF transmembrane conductance regulator (CFTR) dysfunction as well as infection. Mutant DeltaF508 CFTR is mistrafficked, accumulates in the endoplasmic reticulum (ER), and may cause "cell stress" and activation of nuclear factor (NF)-kappaB. G551D mutants also lack Cl-...
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