Scn1a encodes the alpha subunit of a heteromeric complex forming the voltage sensitive sodium channel. Mutations in SCN1A are associated with several epilepsy syndromes including Dravet Syndrome, an infant-onset epileptic encephalopathy. Mice homozygous for this null allele exhibit tremors, ataxia and seizures; death occurs by postnatal day 16. Heterozygous mice on the C57BL/6 background develop spontaneous seizures and die within weeks. On an F1 hybrid (C57BL/6J x 129S6/SvEvTac) background, heterozygotes exhibit an intermediate phenotype including seizures and a 50% lethality by one month of age. On the 129S6/SvEvTac background, mice do not exhibit an overt phenotype; lifespan is normal and no seizures are recorded by EEG monitoring. This strain may be useful for genetic analysis of epilepsy and Dravet syndrome.

In an attempt to offer alleles on well-characterized or multiple genetic backgrounds, alleles are frequently moved to a genetic background different from that on which an allele was first characterized. It should be noted that the phenotype could vary from that originally described. We will modify the strain description if necessary as published results become available.