While we observed a trend toward an increase in ASL height following stimulation of CFTR with vasoactive intestinal peptide (VIP), this increase did not reach statistical significance under any condition when CF sputum was present ( Figures 5 D and S5 B). 26 Only NHBE cells in the presence of normal lung sputum produced a significant increase in ASL height following VIP stimulation (from 8.51 ± 3.27 μm to 16.51 ± 9.41 μm, p < 0.001, n = 9).
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Expression of gain-of-function CFTR in cystic fibrosis airway cells restores epithelial function better than wild-type or codon-optimized CFTR.
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