Figure 1

Functional thermal instability of CFTR-dependent transepithelial current in primary F508del/F508del HAE cells. (a) Schematic representation of a current obtained with the Ussing Chamber technique showing the parameters used to record and analyse CFTR-dependent Isc stability over time. ΔIsc res, residual ΔIsc 35+/− 2 minutes after the peak or Isc stabilization; ΔIsc act, ΔIsc after activation; fsk, forskolin; inh172, CFTR(inh)-172. (b) Representative Ussing Chamber recordings obtained in primary F508del/F508del HAE cells at 29 °C (grey line) or 37 °C (black line) with forskolin + VX-770 (1 μM) addition. (c) Box plot representations showing the distribution of ΔIsc res values normalized to ΔIsc act at 29 °C and 37 °C. **p < 0.01; two-tailed Mann-Whitney test; n = 5–6.