Table 2.

Genes for voltage-gated ion channel subunits with spontaneous epilepsy phenotypes in mice

Gene	Current/protein	Seizure type	Mutation Ko, knock-out Ki, knock-in	Reference
Sodium
Scna1a	Nav1.1 α subunit	convulsive	Ko	Yu et al. 2006; Ogiwara et al. 2007
Scna1a			Targeted R1648H human Ki	Martin et al. 2010
Scna1b			Ko	Chen et al. 2004
Scn2a	Nav1.2 α subunit	convulsive	transgenic GAL879-881QQQ	Kearney et al. 2001
Calcium
Cacna1a	Cav2.1 P/Q-type α subunit	absence	Spontaneous alleles (tottering, leaner, rocker roller, tg4J, tg5J)	Fletcher et al. 1996; Zwingman et al. 2001; Mori et al. 2000; Miki et al. 2008
Cacna1a	Cav2.1 P/Q-type	absence	Cerebellar selective PCP2-Cre promoter Ko	Mark et al. 2011
Cacna1a	Cav2.1 P/Q-type	absence	Ko	Llinas et al. 2007
Cacnb4	β4 regulatory subunit	absence	Spontaneous (lethargic)	Burgess et al. 1997
Cacna1g	Cav3.1 T-type α subunit	absence	Bac transgene overexpression	Ernst et al. 2009
Cacna2d2	α2δ regulatory subunit	absence	Spontaneous several alleles	Barclay et al. 200; Brill et al. 2004
Potassium
Kcna1	Kv1.1	convulsive	Ko	Smart et al. 1998
Kcna1	Kv1.1	absence	Overexpression	Sutherland et al. 1999
Kcna2	Kv1.2	convulsive	Ko	Brew et al. 2007
Kcnh3	Kv12.2	non-convulsive	Ko	Shang et al. 2010
Kcnmb4	β4 regulatory subunit calcium-activated	non-convulsive	Ko	Brenner et al. 2005
Kcnc2	Kv3.2	convulsive	Ko	Lau et al. 2000
KcnJ6	Girk2 ATP sensitive	convulsive	Ko	Signorini et al. 1997
Kcnq1	Kv7.1	convulsive	Human Ki T311I A340E	Goldman et al. 2009
Kcnq2	Kv7.2	convulsive	Human Ki A306T	Singh et al. 2008
Kcnq3	Kv7.3	convulsive	Human Ki G311V	Singh et al. 2008
Cyclic nucleotide gated
Hcn2	Ih hyperpolarization-activated cyclic nucleotide-gated	absence	Ko spontaneous apathetic	Ludwig et al. 2003; Chung et al. 2009
Chloride
Clcn3	Ichloride	convulsive	Ko	Dickerson et al. 2002

Full references can be found in Supplementary information. The neuronal localization of some of the channel proteins is shown in Figure 1.