Loss of A561E-CFTR channel activity in excised inside-out membrane patches. (A–D) Time
courses of Po for wild-type CFTR and low temperature-rescued A561E-CFTR in excised
inside-out membrane patches once channel activation was complete. Low temperature-rescued A561E-CFTR
Cl− channels were studied at 23°C (B), at 37°C (C) and at
37°C in the presence of ivacaftor (VX-770; 10 μM) (D). Po values were
calculated for each 30 s interval. In C and D, the left and right ordinates show Po
(bars) and normalized Po (circles), respectively; Po values were normalized to
that measured immediately temperature reached 37°C (t = 0–30 s). The wild-type
CFTR data are the same as Figure 3A. For A561E-CFTR, the
membrane patch was excised and channels activated at either 23°C (B) or 27°C (C and
D). In B, temperature was maintained at 23°C, whereas in C and D, it was increased to
37°C before Po values were measured. Data are means ± SEM quantified from
4165 ± 1626 transitions (A561E: 23°C, N ≤ 3, n = 4);
6076 ± 2300 transitions (A561E: 37°C, N ≤ 5, n = 6) and
5164 ± 1639 transitions (A561E: 37°C + VX-770, N ≤ 2, n
= 4).