For example, the apparently counterintuitive observation that the Sigma1R antagonist NE-100 reduced epileptiform activity and hyperlocomotion in the preclinical scn1Lab−/− mutant zebrafish model of Dravet syndrome can be explained in terms of this biphasic dose-response behavior [23]. The gene discussed is SIGMAR1; the disease is encephalopathy, progressive, early-onset, with brain edema and/or leukoencephalopathy.