Table 2.
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.