Age-related atrophy and neurodegeneration of BFCNs is recapitulated in mouse models of DS (Holtzman et al., 1992, 1996; Fiedler et al., 1994; Cooper et al., 2001; Granholm et al., 2002) and was attributed to APP gene triplication through disruption of endosomal phenotype and function (Cataldo et al., 2003). This evidence concerns the gene APP and Dravet syndrome.