TABLE 1

Sensitivity of CFTR mutants to potentiators Mean data ± S.E.M. for Kd, maximal current (Imax), and Hill coefficient values obtained by fitting dose-response relationships of CFTR potentiators in Ussing chamber experiments.

Kd
Imax
nH
n
μM μA/cm2
E193K
   PG-01 0.22 ± 0.03 37.4 ± 3.6 1.5 ± 0.2 6
   SF-01 0.74 ± 0.19 20.6 ± 2.7 1.3 ± 0.1 9
   Felodipine 0.67 ± 0.14 32.7 ± 2.4 1.4 ± 0.1 9
G970R
   PG-01 0.45 ± 0.07** 60.2 ± 8.1 1.4 ± 0.2 10
   SF-01 0.45 ± 0.07ns 17.6 ± 2.7 1.6 ± 0.2 10
   Felodipine 2.03 ± 0.39** 75.7 ± 7.4 1.2 ± 0.1 9
G551D
   PG-01 1.94 ± 0.54*† 21.5 ± 4.4 1.4 ± 0.2 8
   SF-01 1.10 ± 0.12ns 5.9 ± 0.7 1.8 ± 0.3 15
   Felodipine
10.22 ± 1.12**††
68.4 ± 5.4
2.2 ± 0.4
9

  • ns, nonsignificant

  • ↵* P < 0.05

  • ↵** P < 0.01; vs. the same potentiator in E193K

  • ↵† P < 0.05

  • ↵†† P < 0.01; vs. the same potentiator in G970R