NR AWIX
AU Legname,G.; Baskakov,I.V.; Nguyen,H.O.B.; Cohen,F.E.; DeArmond,S.J.; Prusiner,S.B.
TI Mouse synthetic prions from full-length prion protein
QU International Conference - Prion 2006: Strategies, advances and trends towards protection of society - 3.10.-6.10.2006, Torino, Italy, Lingotto Conference Centre - Poster sessions S-16
PT Konferenz-Poster
AB We recently described the in vitro production of infectious prions from purified recombinant prion protein of mouse sequence 89 to 230, designated MoPrP(89-230). MoPrP(89-230) was refolded into a ß-sheet-rich amyloid preparation and inoculated into transgenic (Tg) mice expressing PrP of the same sequence. Multiple novel mouse synthetic prion strains were identified, as judged by: (i) incubation times, (ii) a conformational stability assay, and (iii) neuropathologic changes. In another set of experiments, amyloid fibrils were produced using either truncated MoPrP(89-230) or full-length MoPrP(23-230). These preparations were injected into Tg mice expressing full-length MoPrP(23-231). Mice inoculated with either amyloid preparation developed prion disease in 600 days. That prions could be generated in vitro supports the hypothesis that PrPsc is the sole component of the infectious agent responsible for the mammalian prion diseases.
AD G. Legname: Institute for Neurodegenerative Diseases, Department of Neurology, University of California, San Francisco, CA, USA; I.V. Baskakov: 5University of Maryland Biotechnology Institute, Baltimore, MD, USA; H.O.-B. Nguyen: Institute for Neurogenerative Diseases, University of California, San Francisco, CA, USA; F.E. Cohen: Institute for Neurodegenerative Diseases, Department of Biochemistry and Biophysics, University of California, San Francisco, CA, USA; S.J. DeArmond: Institute for Neurodegenerative Diseases, Department of Pathology, University of California, San Francisco, CA, USA; S.B. Prusiner: Institute for Neurodegenerative Diseases, Departments of Neurology, Biochemistry and Biophysics, University of California, San Francisco, CA, USA. E-mail: glegname@ind.ucsf.edu
SP englisch
PO Italien