Skip to main content
. 2020 Sep 4;8:536. doi: 10.3389/fped.2020.00536

Figure 7.

Figure 7

CFTR, ENaC, and CaCC activities of EpiXTM expanded CF patient cultures. (A) The F508del/R117H-7H genotype was used as an internal reference to compare CFTR function among genotypes. CFTR function was quantified from the forskolin plus VX-770 stimulated current that was blockable by CFTR inhibitors and in cultures that received no CFTR corrector treatment. (B) Corresponding ENaC activity was quantified by measuring the change in current before and after addition of amiloride. Note that ENaC activity was significantly increased in cultures with the F508del/F508del or R334W/406-1G->A genotype. (C) Corresponding CaCC activity was quantified by measuring the change in current before addition of ATP and the maximal current stimulated by ATP (peak). Note that ATP induced a decrease in Isc in cultures with the F508del/F508del genotype suggesting that other conductances dominated the secretory response to ATP. All other cultures showed a chloride secretory response to ATP. Bars represent mean ± SE (n = 2–13 experiments per genotype). *denotes significantly different from F508del/R117H control group (Holm-Sidak, p < 0.05).