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. 2004 Jul 30;305(5684):673-6.
doi: 10.1126/science.1100195.

Synthetic mammalian prions

Giuseppe Legname  1 , Ilia V Baskakov, Hoang-Oanh B Nguyen, Detlev Riesner, Fred E Cohen, Stephen J DeArmond, Stanley B Prusiner
Affiliations

Synthetic mammalian prions

Giuseppe Legname et al. Science. .

Abstract

Recombinant mouse prion protein (recMoPrP) produced in Escherichia coli was polymerized into amyloid fibrils that represent a subset of beta sheet-rich structures. Fibrils consisting of recMoPrP(89-230) were inoculated intracerebrally into transgenic (Tg) mice expressing MoPrP(89-231). The mice developed neurologic dysfunction between 380 and 660 days after inoculation. Brain extracts showed protease-resistant PrP by Western blotting; these extracts transmitted disease to wild-type FVB mice and Tg mice overexpressing PrP, with incubation times of 150 and 90 days, respectively. Neuropathological findings suggest that a novel prion strain was created. Our results provide compelling evidence that prions are infectious proteins.

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