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Isolation of infectious, non-fibrillar and oligomeric prions from a genetic prion disease

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Gerstmann-Sträussler-Scheinker disease (GSS) is characterized by atypical N- and C-terminal cleaved protease-resistant PrPSc. Vanni et al. report that PrPSc oligomers purified from patients with GSS are authentic – but non-fibrillar… Click to show full abstract

Gerstmann-Sträussler-Scheinker disease (GSS) is characterized by atypical N- and C-terminal cleaved protease-resistant PrPSc. Vanni et al. report that PrPSc oligomers purified from patients with GSS are authentic – but non-fibrillar – prions, thus uncovering the minimal prion protein domain able to encode infectious prions.

Keywords: isolation infectious; prion; non fibrillar; disease; infectious non

Journal Title: Brain
Year Published: 2020

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