Considering the importance of GABA signaling in epilepsy and reported dysfunction in GABAergic signaling in Dravet syndrome [14–16], Gabra2 was identified as the top candidate modifier gene at Dsm1. Expression of Gabra2 was significantly elevated in wildtype 129 mice compared to [129XB6]F1 mice (Fig 2B). The gene discussed is SRSF8; the disease is encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.