BACE2 and Dravet syndrome: Due in part to the triplication of genes encoding amyloid precursor protein (APP) and β-amyloid cleavage enzyme 2 (BACE2) (St George-Hyslop et al., 1987; Acquati et al., 2000), located on chromosome 21, individuals with DS display a progressive accumulation of amyloid-beta (Aβ) peptides starting before birth (Lemere et al., 1996; Teller et al., 1996; Mori et al., 2002; Iulita et al., 2014).