We recently demonstrated in an animal model that selective deletion of Ube3a in GABAergic neurons causes both circuit hyperexcitability and seizures; the presence of UBE3A in inhibitory neurons of the human neocortex further supports the hypothesis that GABAergic neurons play a key role in precipitating the electroencephalographic abnormalities and epilepsy seen in AS. This evidence concerns the gene UBE3A and epilepsy.