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We have established aDrosophilamodel of Gerstmann–Sträussler–Scheinker (GSS) syndrome by expressing mouse prion protein (PrP) having leucine substitution at residue 101 (MoPrPP101L). Flies expressing MoPrPP101L, but not wild-type MoPrP (MoPrP3F4), showed severe defects in climbing ability and early death. Expressed MoPrPP101LinDrosophilawas differentially glycosylated, localized at the synaptic terminals and mainly present as deposits in adult brains. We found that behavioral defects and early death of MoPrPP101Lflies were not due to Caspase 3-dependent programmed cell death signaling. In addition, we found that Type 1 glutamatergic synaptic boutons in larval neuromuscular junctions of MoPrPP101Lflies showed significantly increased numbers of satellite synaptic boutons. Furthermore, the amount of Bruchpilot and Discs large in MoPrPP101Lflies was significantly reduced. Brains from scrapie-infected mice showed significantly decreased ELKS, an active zone matrix marker compared with those of age-matched control mice. Thus, altered active zone structures at the molecular level may be involved in the pathogenesis of GSS syndrome inDrosophilaand scrapie-infected mice.

A Drosophila model of GSS syndrome suggests defects in active zones are responsible for pathogenesis of GSS syndrome