Article
Gerstmann-Sträussler-Scheinker disease subtypes efficiently transmit in bank voles as genuine prion diseases.
Scientific reports - 4 Feb 2016
Pirisinu Laura, Di Bari Michele A, D'Agostino Claudia, Marcon Stefano, Riccardi Geraldina, Poleggi Anna, Cohen Mark L, Appleby Brian S, Gambetti Pierluigi, Ghetti Bernardino, Agrimi Umberto, Nonno Romolo
Abstract excerpt
Gerstmann-Sträussler-Scheinker disease (GSS) is an inherited neurodegenerative disorder associated with mutations in the prion protein gene and accumulation of misfolded PrP with protease-resistant fragments (PrP(res)) of 6-8 kDa. With the exception of a few GSS cases characterized by co-accumulation of PrP(res) of 21 kDa, efforts to transmit GSS to rodents have been unsuccessful. As a result, GSS subtypes...
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