The K328M substitution in the human GABAA receptor gamma2 subunit causes GEFS+ and premature sudden death in knock-in mice

Abstract

• Gabrg2 +/K328M KI mice have febrile seizures and display multiple seizure types in adulthood. • Gabrg2 +/K328M KI mice display hyperactivity and premature sudden death. • Gabrg2 +/K328M KI mice have decreased amplitude of mIPSCs in somatosensory cortical layer V/VI pyramidal neurons. • Gabrg2 +/K328M KI mice have prolonged spontaneous thalamocortical oscillations.