Figure 4.
CFTR function measurements in EpiXTM-expanded non-CF cultures. Normal bronchial epithelial cells, one donor, passage 1. (A) Original short circuit current trace, untreated. Transepithelial chloride current (ICl) was recorded in presence of amiloride. Stimulation of wildtype CFTR currents by the cAMP agonist forskolin (20 μM, serosal) and CFTR potentiator VX-770 (1 μM, mucosal) and subsequent inhibition by CFTR inhibitors (CFTRinh-172, PPQ-102, GlyH-101; 50 μM, mucosal). Note, trace does not show initial amiloride and final ATP additions. (B) Individual responses. Baseline represents change in cAMP-dependent CFTR current by forskolin alone. VX-770 represents change in current by forskolin+VX-770. * denotes significant increase by VX-770 (paired t-test, p < 0.05, n = 12). (C) CFTR-specific currents represent change in current due to stimulation by forskolin (cAMP) and VX-770 (upward bar) and total inhibition of cAMP-stimulated and cAMP+VX-770 stimulated CFTR currents by CFTR inhibitors (downward bar). Note that 100% normal CFTR activity is defined as the blocked forskolin-stimulated CFTR activity. Bars represent mean ± SE (n = 12).