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B in airway epithelial cells is dependent
on CFTR trafficking and Cl
channel function
College of Physicians and Surgeons, Columbia University, New York, New York 10032
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 
F508 CFTR is mistrafficked, accumulates in the endoplasmic reticulum (ER), and may cause "cell stress" and activation of nuclear factor (NF)-
B. G551D mutants also lack Cl
channel function, but CFTR is trafficked
normally. We compared the effects of CFTR mutations on the endogenous
activation of an NF-B reporter construct. In transfected Chinese
hamster ovary cells, the mistrafficked F508 allele caused a
sevenfold activation of NF-B compared with wild-type CFTR or the
G551D mutant (P < 0.001). NF-B was also activated
in 9/HTEo/pCep-R cells and in 16HBE/pcftr
antisense cell lines, which lack CFTR Cl channel function
but do not accumulate mutant protein in the ER. This endogenous
activation of NF-B was associated with elevated interleukin-8
expression. Impaired CFTR Cl channel activity as well as
cell stress due to accumulation of mistrafficked CFTR in the ER
contributes to the endogenous activation of NF-B in cells with the
CFTR mutation.
nuclear factor-B; cystic fibrosis transmembrane conductance
regulator; chloride channel; inflammatory response; intracellular
calcium; mitogen-activated protein kinase
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