Mutations in inhibitory GABAA receptor subunit genes (GABRA1, GABRB3, GABRG2, and GABRD) of human have been associated with genetic epilepsy syndromes including childhood absence epilepsy, juvenile myoclonic epilepsy, pure febrile seizures, generalized epilepsy with febrile seizures plus, and Dravet syndrome (DS)/severe myoclonic epilepsy in infancy. Here, GABRB3 is linked to Dravet syndrome.