Figure 6.
Revertant mutations modify F508del‐CFTR channel gating. (A) Representative single‐channel recordings of the indicated CFTR constructs in excised inside‐out membrane patches from BHK cells. ATP (1 mM) and PKA (75 nM) were continuously present in the intracellular solution. Dotted lines indicate the closed channel state and downward deflections correspond to channel openings. (B–D) Po, MBD and IBI for the indicated CFTR constructs. Data are means ± SEM (WT, n = 5; F508del, n = 18; F508del‐G550E, Po, n = 7, MBD and IBI, n = 4; F508del‐R1070W, Po, n = 5, MBD and IBI, n = 4; F508del‐G550E‐R1070W, Po, n = 6, MBD and IBI, n = 4); *, P < 0.05 versus WT‐CFTR; †, P < 0.05 versus F508del‐CFTR. In (B–D), the wild‐type and F508del‐CFTR data are the same as Figure 3. Other details as in Figure 3.