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Science 20 December 1996:
Vol. 274. no. 5295, pp. 2079 - 2082
DOI: 10.1126/science.274.5295.2079
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Reports

Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity

Glenn C. Telling, Piero Parchi, Stephen J. DeArmond, Pietro Cortelli, Pasquale Montagna, Ruth Gabizon, James Mastrianni, Elio Lugaresi, Pierluigi Gambetti, Stanley B. Prusiner *

The fundamental event in prion diseases seems to be a conformational change in cellular prion protein (PrPC) whereby it is converted into the pathologic isoform PrPSc. In fatal familial insomnia (FFI), the protease-resistant fragment of PrPSc after deglycosylation has a size of 19 kilodaltons, whereas that from other inherited and sporadic prion diseases is 21 kilodaltons. Extracts from the brains of FFI patients transmitted disease to transgenic mice expressing a chimeric human-mouse PrP gene about 200 days after inoculation and induced formation of the 19-kilodalton PrPSc fragment, whereas extracts from the brains of familial and sporadic Creutzfeldt-Jakob disease patients produced the 21-kilodalton PrPSc fragment in these mice. The results presented indicate that the conformation of PrPSc functions as a template in directing the formation of nascent PrPSc and suggest a mechanism to explain strains of prions where diversity is encrypted in the conformation of PrPSc.

G. C. Telling and J. Mastrianni, Department of Neurology, University of California, San Francisco, CA 94143, USA.
P. Parchi and P. Gambetti, Division of Neuropathology, Department of Pathology, Case Western Reserve University, Cleveland, OH 44106, USA.
S. J. DeArmond, Departments of Neurology and Pathology, University of California, San Francisco, CA 94143, USA.
P. Cortelli, P. Montagna, E. Lugaresi, Department of Neurology, University of Bologna, Bologna 40123, Italy.
R. Gabizon, Department of Neurology, Hadassah Medical Center, Hebrew University, Jerusalem 91120, Israel.
S. B. Prusiner, Departments of Neurology and Biochemistry and Biophysics, University of California, San Francisco, CA 94143, USA.
*   To whom correspondence should be addressed at the Department of Neurology, HSE-781, University of California, San Francisco, CA 94143-0518, USA.

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Glycosylation differences between the normal and pathogenic prion protein isoforms.
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Prion Protein Conformation in a Patient with Sporadic Fatal Insomnia.
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   Abstract »
Strain-dependent Differences in beta -Sheet Conformations of Abnormal Prion Protein.
B. Caughey, G. J. Raymond, and R. A. Bessen (1998)
J. Biol. Chem. 273, 32230-32235
   Abstract »    Full Text »    PDF »
Prions.
S. B. Prusiner (1998)
PNAS 95, 13363-13383
   Abstract »    Full Text »    PDF »
Prions.