Synthetic Mammalian Prions-Reference-Cited by-同舟云学术

Synthetic Mammalian Prions

Published:2004-07-30 Issue:5684 Volume:305 Page:673-676
ISSN:0036-8075
Container-title:Science
language:en
Short-container-title:Science

Author:

Legname Giuseppe¹²³⁴⁵,Baskakov Ilia V.¹²³⁴⁵,Nguyen Hoang-Oanh B.¹²³⁴⁵,Riesner Detlev¹²³⁴⁵,Cohen Fred E.¹²³⁴⁵,DeArmond Stephen J.¹²³⁴⁵,Prusiner Stanley B.¹²³⁴⁵

Affiliation:

1. Institute for Neurodegenerative Diseases, University of California, San Francisco, CA 94143, USA.

2. Department of Neurology, University of California, San Francisco, CA 94143, USA.

3. Department of Cellular and Molecular Pharmacology, University of California, San Francisco, CA 94143, USA.

4. Department of Biochemistry and Biophysics, University of California, San Francisco, CA 94143, USA.

5. Department of Pathology, University of California, San Francisco, CA 94143, USA.

Abstract

Recombinant mouse prion protein (recMoPrP) produced in Escherichia coli 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.

Publisher

American Association for the Advancement of Science (AAAS)

Subject

Multidisciplinary

Reference44 articles.

1. S. B. Prusiner, in Prion Biology and Diseases, S. B. Prusiner, Ed. (Cold Spring Harbor Laboratory Press, Cold Spring Harbor, NY, 2004), pp. 89–141.

2. A Change in the Conformation of Prions Accompanies the Emergence of a New Prion Strain

3. High-Level Expression and Characterization of a Purified 142-Residue Polypeptide of the Prion Protein

4. Protease-resistant prion protein produced in vitro lacks detectable infectivity.

5. Serial transmission in rodents of neurodegeneration from transgenic mice expressing mutant prion protein.

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