Article
Mutant prion protein expression causes motor and memory deficits and abnormal sleep patterns in a transgenic mouse model.
Neuron - 26 Nov 2008
Dossena Sara, Imeri Luca, Mangieri Michela, Garofoli Anna, Ferrari Loris, Senatore Assunta, Restelli Elena, Balducci Claudia, Fiordaliso Fabio, Salio Monica, Bianchi Susanna, Fioriti Luana, Morbin Michela, Pincherle Alessandro, Marcon Gabriella, Villani Flavio, Carli Mirjana, Tagliavini Fabrizio, Forloni Gianluigi, Chiesa Roberto
Abstract excerpt
A familial form of Creutzfeldt-Jakob disease (CJD) is linked to the D178N/V129 prion protein (PrP) mutation. Tg(CJD) mice expressing the mouse homolog of this mutant PrP synthesize a misfolded form of the mutant protein, which is aggregated and protease resistant. These mice develop clinical and pathological features reminiscent of CJD, including motor dysfunction, memory impairment, cerebral PrP deposition, and...
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