Table 1.
Electrophysiological properties of Kv11.1 missense mutations.
| LQT2 | Current Density | Activation | Deactivation @ −50mV | Inactivation @ 0mV | ||
|---|---|---|---|---|---|---|
|
| ||||||
| pA/pF | V1/2 (mv) | slope (mv/e-fold) | tau slow (s) | tau fast (ms) | tau (ms) | |
| WT | 97.7±6.2 (9) | −8.3±0.4 (5) | 6.5 ±0.4 (5) | 5.3±0.3 (5) | 835±32 (5) | 12.4±0.7 (3) |
| D16A | 57.3±12.4 (4) | 4.9±2.5* (4) | 6.9±0.4 (4) | 9.3±0.8* (4) | 1281±105* (4) | 17.7± 0.1* (4) |
| R20G | 48.5±7.7 (4) | −15.0±1.2 (4) | 6.6±0.2 (4) | 6.1±0.1 (4) | 943±119 (4) | 17.8±1.8* (4) |
| E58D | 62.2±9.7 (8) | −8.8±1.5 (7) | 7.5±0.3 (7) | 4.9±0.3 (8) | 746±37 (8) | 16.1±0.6* (4) |
| V115M | 73.1±15.3 (9) | −9.2±0.7 (8) | 6.8±0.2 (8) | 5.1±0.5 (4) | 863±48 (4) | 12.7±0.1 (3) |
| M124R | 21.6±5.3 (7) | −7.3±1.6 (4) | 7.6±0.3 (4) | 2.2±0.1* (5) | 430±28* (5) | n/a |
| F125C | 54.1±13.7 (9) | −11.2±1.4 (9) | 6.7±0.1 (9) | 3.4±0.2 (9) | 615±33 (9) | 13.0±0.5 (4) |
| E130K | 43.2±6.1 (7) | −19.4±1.9* (9) | 6.6±0.3 (9) | 6.9±0.7 (7) | 922±65 (7) | 10.4±0.3 (6) |
| S706C | 97.7±6.2 (6) | −14.4±2.3 (5) | 6.6±0.4 (5) | 5.6±0.7 (5) | 914±65 (5) | 13.4±1.6 (3) |
| I711V | 45.5±4.0 (4) | −5.9±1.8 (4) | 7.6±0.6 (4) | 3.2±0.2* (4) | 512±16* (4) | n/a |
| D767Y | 73.1±9.7 (5) | −21.0±1.2* (5) | 6.1±0.2 (5) | 3.8±0.3 (4) | 741±82 (4) | 11.4±0.4 (4) |
| R791W | 105±11 (4) | −20.5±1.4* (4) | 6.1±0.2 (4) | 3.1±0.6* (4) | 620±104 (4) | 16.0±2.6 (4) |
| R835W | 17.2±1.8 (5) | 7.8±1.6* (5) | 9.0±0.6* (5) | 1.9±0.2* (4) | 416±31* (4) | 12.6±0.2 (3) |
| R1005Q | 97.7±6.2 (7) | −14.6±0.5* (7) | 6.4±0.3 (7) | 4.7±0.3 (6) | 823±43 (6) | 15.1±0.6 (5) |
| L1049P | 34.6±4.5 (7) | −17.0±2.5* (5) | 7.7±0.6 (5) | 11.3±1.0* (5) | 1440±24* (5) | 10.6+0.5 (5) |
| L1066V | 74.3±8.7 (6) | −0.5±0.8* (5) | 7.1±0.4 (5) | 4.0±0.2 (4) | 719±29 (4) | 18.9±1.1* (4) |
| V644L | 62.0±6.4 (12) | −10.6±1.7 (7) | 6.6±0.4 (7) | 4.8±0.3 (4) | 790±87 (4) | 8.9±0.3* (5) |
| I662T | 73.1±10.0 (8) | −8.4±1.0 (6) | 7.3±0.1 (6) | 7.5±0.6 (5) | 822±57 (5) | 10.6±0.6 (4) |
Values with an asterisk indicate statistical significance (P<0.05) compared with WT assessed using one-way analysis of variance followed by the Tukey post hoc test.