TABLE 1.
Biophysical parameters for activation, fast inactivation and slow inactivation of WTs and mutants of NaV1.1 channel.
| Channel |
Activation (mV) |
Fast inactivation (mV) |
Slow inactivation (mV) |
tauslow (ms) at −10 mV |
References | ||||
| V1/2 | k | V1/2 | k | V1/2 | k | experiment | model | ||
| Familial hemiplegic migraine type 3 (FHM3) | |||||||||
| L263V | −24.6 | 7.1 | −54.4 | 6.7 | −54.1 | 5.0 | 3,507 | 3,500 | Kahlig et al., 2008 |
| gain-of-function/WT | −21.5 | 7.2 | −62.2 | 6.4 | −66.8 | 6.3 | 2,100 | 2,100 | |
| Q1478K combined | −24.8 | 9.1 | −60.1 | 7.4 | −77.2 | 7.7 | 938 (−5 mV) | 940 (−5 mV) | Cestele et al., 2008 |
| gain- and loss-of function/WT | −24.5 | 7.9 | −65.1 | 6.0 | −75.8 | 6.2 | 2.090 (−5 mV) | 2,100 (−5 mV) | |
| L1649Q | −22.9 | 7.4 | −34.5 | 7.1 | −57.7 | 8.9 | 1,885 (0 mV) | 1,885 (0 mV) | Cestele et al., 2013 |
| gain-of-function/WT | −21.0 | 6.6 | −54.2 | 5.5 | −59.7 | 9.7 | 2,616 (0 mV) | 2,600 (0 mV) | |
| L1670W | −16.8 | 6.1 | −47.3 | 6.1 | −34.8 | 10.0 | 2,070 | 2,070 | Dhifallah et al., 2018 |
| gain-of-function/WT | −21.6 | 6.6 | −55.6 | 4.9 | −53.5 | 7.6 | 1,310 | 1,300 | |
| Familial simple febrile seizures | |||||||||
| M145T | −11.7 | 7.1 | −65.5 | 9.1 | – | – | – | Mantegazza et al., 2005 | |
| loss-of-function/WT | −21.7 | 6.15 | −64.9 | 8.0 | |||||
| Generalized epilepsy with febrile seizures plus (GEFS +) | |||||||||
| R1648H | −19.9 | 8.5 | −61.3 | 7.8 | −68.6 | 6.1 | 3,112 | 3,112 | Kahlig et al., 2006 |
| gain-of-function/WT | −19.4 | 7.9 | −62.7 | 6.9 | −66.7 | 7.8 | 3,029 | 3,000 | |
V1/2 is the voltage of half-maximal activation or inactivation; k is a slope factor; tauslow is kinetics of the development of slow inactivation. Voltage indicated in brackets shows values at which the recordings were performed in the quoted papers and in model studies.