Table 1.
Experimental data on biophysical effects of the E161K mutation in SCN5A.
Steady-state activation |
Steady-state inactivation |
|||||||||
---|---|---|---|---|---|---|---|---|---|---|
Study | Expression system | Background | Channel type | Peak current density (nA/pF) | V1/2 (mV) | k (mV) | n | V1/2 (mV) | k (mV) | n |
Experimental Data | ||||||||||
Smits et al., 2005 | tsA201 cells | H558 | Wild-type | 0.71 ± 0.11 | -42.6 ± 1.4 | 6.7 ± 0.4 | 9 | -89.4 ± 1.2 | -4.9 ± 0.3 | 9 |
E161K | 0.28 ± 0.05∗ | -30.7 ± 0.8∗ | 7.9 ± 0.3∗ | 13 | -88.5 ± 0.9 | -4.4 ± 0.1 | 13 | |||
Gui et al., 2010a | HEK-293 cells | H558 | Wild-type | 0.87 ± 0.08 | -34.7 ± 0.7 | 6.9 ± 0.2 | 23 | -81.4 ± 0.7 | -6.4 ± 0.1 | 24 |
E161K | 0.20 ± 0.03∗ | -14.9 ± 0.7∗ | 9.0 ± 0.1∗ | 19 | -79.9 ± 0.9 | -6.6 ± 0.1 | 21 | |||
Gui et al., 2010b | HEK-293 cells | H558 | Wild-type | 0.81 ± 0.08 | -33.8 ± 0.7 | 6.7 ± 0.2 | 11 | -80.3 ± 0.9 | -6.2 ± 0.1 | 12 |
E161K | 0.19 ± 0.03∗ | -14.7 ± 0.6∗ | 8.9 ± 0.1∗ | 11 | -78.4 ± 0.8 | -6.5 ± 0.2 | 12 | |||
R558 | Wild-type | 0.92 ± 0.17 | -35.5 ± 1.3 | 6.8 ± 0.2 | 9 | -81.8 ± 1.0 | -6.5 ± 0.1 | 10 | ||
E161K | 0.15 ± 0.02∗ | -20.9 ± 1.2∗ | 8.0 ± 0.1∗ | 14 | -88.8 ± 1.8∗ | -6.7 ± 0.2 | 10 | |||
Model Parameters | ||||||||||
Fabbri et al., 2017 | -30.5 | 6.5 | -69.8 | -4.5 | ||||||
Maleckar et al., 2009 | -15.7 | 6.4 | -63.6 | -5.3 | ||||||
All experimental data obtained at room temperature and expressed as mean ± SEM. ∗P < 0.05 vs. wild-type.