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- Title
Synthetic mammalian prions.
- Authors
Legname, Giuseppe; Baskakov, Ilia V; Nguyen, Hoang-Oanh B; Riesner, Detlev; Cohen, Fred E; DeArmond, Stephen J; Prusiner, Stanley B
- 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.
- Publication
Science (New York, N.Y.), 2004, Vol 305, Issue 5684, p673
- ISSN
1095-9203
- Publication type
Journal Article
- DOI
10.1126/science.1100195