Table 1. Comparison of Na V 1.2 L1563V... | Rockefeller University Press

Table 1.

Comparison of Na_V1.2 L1563V and R1882Q electrophysiology studies

Disease	SCN2A α subunit			Experimental conditions		Channel electrophysiological properties						Reference
Disease	Mutation	Species	Isoform	Exogenous β1/β2 subunit	Cell type	Current density	V-dependent activation	V-dependent inactivation	Fast inactivation time constant τ	Persistent inward current	Recovery from inactivation	Reference
BFNIS	L1563V	Human	Neonatal	Neither	tsA201	Unchanged	Unchanged	Depolarized	Greater	Unchanged	Quicker	Xu et al., 2007
			Adult	Neither	tsA201	Unchanged						Xu et al., 2007
			Adult	β1 and β2	tsA201	Decreased	Unchanged	Depolarized	Greater	Unchanged	Quicker	Misra et al., 2008
			Adult	Neither	Chinese hamster ovarian cells	Unchanged	Depolarized	Depolarized	Like the adult WT	Unchanged	Quicker	Berecki et al., 2018
			Adult	β1 and β2	HEK293T	Unchanged	Unchanged	Depolarized	Like the adult WT	Unchanged	Unchanged	Begemann et al., 2019
EIMFS	R1882Q	Human	Adult	Neither	Chinese hamster ovarian cells	Increased	Hyperpolarized	Depolarized	Greater	Increased	Unchanged	Berecki et al., 2018
			Neonatal	β1 and β2	tsA201	Unchanged	Unchanged	Depolarized	Greater	Unchanged	Unchanged	Thompson et al., 2020
			Adult	β1 and β2	tsA201	Unchanged	Depolarized	Depolarized	Greater	Unchanged	Unchanged	Thompson et al., 2020

Disease	SCN2A α subunit			Experimental conditions		Channel electrophysiological properties						Reference
Disease	Mutation	Species	Isoform	Exogenous β1/β2 subunit	Cell type	Current density	V-dependent activation	V-dependent inactivation	Fast inactivation time constant τ	Persistent inward current	Recovery from inactivation	Reference
BFNIS	L1563V	Human	Neonatal	Neither	tsA201	Unchanged	Unchanged	Depolarized	Greater	Unchanged	Quicker	Xu et al., 2007
			Adult	Neither	tsA201	Unchanged						Xu et al., 2007
			Adult	β1 and β2	tsA201	Decreased	Unchanged	Depolarized	Greater	Unchanged	Quicker	Misra et al., 2008
			Adult	Neither	Chinese hamster ovarian cells	Unchanged	Depolarized	Depolarized	Like the adult WT	Unchanged	Quicker	Berecki et al., 2018
			Adult	β1 and β2	HEK293T	Unchanged	Unchanged	Depolarized	Like the adult WT	Unchanged	Unchanged	Begemann et al., 2019
EIMFS	R1882Q	Human	Adult	Neither	Chinese hamster ovarian cells	Increased	Hyperpolarized	Depolarized	Greater	Increased	Unchanged	Berecki et al., 2018
			Neonatal	β1 and β2	tsA201	Unchanged	Unchanged	Depolarized	Greater	Unchanged	Unchanged	Thompson et al., 2020
			Adult	β1 and β2	tsA201	Unchanged	Depolarized	Depolarized	Greater	Unchanged	Unchanged	Thompson et al., 2020