The GABRG2 Q390X knock-in mice displayed spontaneous seizure and neurobehavioral comorbidities that were observed in Dravet Syndrome, and molecular mechanism study demonstrated that the Q390X mutation, which terminated the subunit before the transmembrane helix 4, caused the aggregation of the variant and reduced miniature IPSCs [51]. Here, GABRG2 is linked to encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.