The P264LfsTer10 mutation is reminiscent of the megencephaly (mceph) mouse model of Kcna1, which has epilepsy due to an 11-nucleotide deletion causing a frameshift that truncates the Kv1.1 protein at amino acid 230 in the S2 domain [89,90,91,92]. Here, KCNA1 is linked to epilepsy.