Synthetic Mammalian Prions

DOI Web Site 被引用文献27件
  • Giuseppe Legname
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Ilia V. Baskakov
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Hoang-Oanh B. Nguyen
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Detlev Riesner
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Fred E. Cohen
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Stephen J. DeArmond
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.
  • Stanley B. Prusiner
    Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.

抄録

<jats:p> Recombinant mouse prion protein (recMoPrP) produced in <jats:italic>Escherichia coli</jats:italic> was polymerized into amyloid fibrils that represent a subset of β 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. </jats:p>

収録刊行物

  • Science

    Science 305 (5684), 673-676, 2004-07-30

    American Association for the Advancement of Science (AAAS)

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