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Publication : Synthetic mammalian prions.

First Author  Legname G Year  2004
Journal  Science Volume  305
Issue  5684 Pages  673-6
PubMed ID  15286374 Mgi Jnum  J:92426
Mgi Id  MGI:3052607 Doi  10.1126/science.1100195
Citation  Legname G, et al. (2004) Synthetic mammalian prions. Science 305(5684):673-6
abstractText  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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