Synthetic mammalian prions.

نویسندگان

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

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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عنوان ژورنال:
  • Science

دوره 305 5684  شماره 

صفحات  -

تاریخ انتشار 2004