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. |