Figure 1.
Comparison of basal CFTR-mediated Cl− secretion in primary human nasal epithelial cells (pHNEs) and rectal biopsies. Measurements of basal CFTR-mediated Cl− secretion in pHNEs (left panels) in samples from one healthy control and five PwCF with different genotypes show similarities with the basal CFTR-mediated Cl− secretion measured in rectal biopsies (right panels) from the same individual. The sample code and genotypes are indicated above the tracings. (A,C,E,G,I,K) Original Ussing chamber (open-circuit) recordings showing transepithelial voltage measurements (Vte) obtained for pHNE monolayers with different CFTR genotypes, after cAMP-dependent activation. Amiloride (20 μM) was kept during the whole experiment duration to avoid interference of ENaC-mediated currents. Lumen-negative transepithelial Vte deflections are observed following luminal stimulation by forskolin alone (Fsk, 2 μM) or together with potentiator ivacaftor (VX770, 3 μM). The latter is fully reverted by application of the specific CFTR inhibitor CFTRInh-172 (30 μM). (B,D,F,H,J,L) Representative original recordings of the effects of cholinergic (by carbachol (CCH), 100 μM, basolateral) and cAMP-dependent (by IBMX/Fsk (I/F), 100 μM/2 μM, basolateral) Cl− secretion on transepithelial Vte in rectal biopsies.