Table 3. ELIC WT and mutant channel properties by patch-clamping giant liposomes composed of 25 mole% POPG (n = 3–7, ± SD).
Desensitization | Activation | Cysteamine response | ||
---|---|---|---|---|
Weighted τ (s) | Norm current at 20 s | τ (ms) | EC50 (mM cysteamine) | |
WT | 1.95 ± 0.48 | 0.100 ± 0.041 | 134 ± 50 | 5.1 ± 1.2 |
R117Q | 1.49 ± 0.40 | 0.060 ± 0.020 | 54 ± 40 * | 3.3 ± 0.9 * |
R123Q | 1.20 ± 0.39 | 0.067 ± 0.015 | 112 ± 65 | 3.7 ± 0.9 |
R286Q | 1.67 ± 0.24 | 0.091 ± 0.023 | 106 ± 34 | 4.3 ± 0.4 |
R299Q | 2.2 ± 0.17 | 0.074 ± 0.008 | 71 ± 27 | 4.7 ± 0.3 |
R301Q | 0.66 ± 0.28 ** | 0.045 ± 0.017 ** | 72 ± 15 | 5.2 ± 0.8 |
R117Q/R299Q | 1.13 ± 0.24 * | 0.025 ± 0.004 ** | 54 ± 26 * | 2.4 ± 0.7 ** |
R123Q/R299Q | 1.66 ± 0.17 | 0.041 ± 0.005 ** | 74 ± 35 | 3.7 ± 0.6 |
R286Q/R299Q | 2.08 ± 0.65 | 0.103 ± 0.032 | 92 ± 27 | 4.7 ± 1.5 |
R301Q/R299Q | 0.45 ± 0.28 ** | 0.030 ± 0.017 ** | 71 ± 21 | 3.9 ± 0.9 |
L238A | 5.90 ± 1.37 ** | 0.374 ± 0.045 ** | 113 ± 51 | 4.9 ± 1.1 |