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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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Branched Polyamines Cure Prion-Infected Neuroblastoma Cells.
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Expression of unglycosylated mutated prion protein facilitates PrPSc formation in neuroblastoma cells infected with different prion strains.
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Genetic influence on the structural variations of the abnormal prion protein.
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Creutzfeldt-Jakob disease with a novel four extra-repeat insertional mutation in the PrP gene.
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   Abstract »    Full Text »    PDF »
Copper(II)-induced Conformational Changes and Protease Resistance in Recombinant and Cellular PrP. EFFECT OF PROTEIN AGE AND DEAMIDATION.
K. Qin, D.-S. Yang, Y. Yang, M. A. Chishti, L.-J. Meng, H. A. Kretzschmar, C. M. Yip, P. E. Fraser, and D. Westaway (2000)
J. Biol. Chem. 275, 19121-19131
   Abstract »    Full Text »    PDF »
Dominant-Negative Inhibition of Prion Formation Diminished by Deletion Mutagenesis of the Prion Protein.
L. Zulianello, K. Kaneko, M. Scott, S. Erpel, D. Han, F. E. Cohen, and S. B. Prusiner (2000)
J. Virol. 74, 4351-4360
   Abstract »    Full Text »
From the Cover: Compelling transgenetic evidence for transmission of bovine spongiform encephalopathy prions to humans.
M. R. Scott, R. Will, J. Ironside, H.-O. B. Nguyen, P. Tremblay, S. J. DeArmond, and S. B. Prusiner (1999)
PNAS 96, 15137-15142
   Abstract »    Full Text »    PDF »
Glycosylation differences between the normal and pathogenic prion protein isoforms.
P. M. Rudd, T. Endo, C. Colominas, D. Groth, S. F. Wheeler, D. J. Harvey, M. R. Wormald, H. Serban, S. B. Prusiner, A. Kobata, et al. (1999)
PNAS 96, 13044-13049
   Abstract »    Full Text »    PDF »
Type 1 protease resistant prion protein and valine homozygosity at codon 129 of PRNP identify a subtype of sporadic Creutzfeldt-Jakob disease.
B. B Worrall, S. T Herman, S. Capellari, T. Lynch, S. Chin, P. Gambetti, and P. Parchi (1999)
J. Neurol. Neurosurg. Psychiatry 67, 671-674
   Abstract »    Full Text »
Protease-resistant and Detergent-insoluble Prion Protein Is Not Necessarily Associated with Prion Infectivity.
G. M. Shaked, G. Fridlander, Z. Meiner, A. Taraboulos, and R. Gabizon (1999)
J. Biol. Chem. 274, 17981-17986
   Abstract »    Full Text »    PDF »
Prion Protein Conformation in a Patient with Sporadic Fatal Insomnia.
J. A. Mastrianni, R. Nixon, R. Layzer, G. C. Telling, D. Han, S. J. DeArmond, and S. B. Prusiner (1999)
N. Engl. J. Med. 340, 1630-1638
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Molecular Genetics of Transmissible Spongiform Encephalopathies.
C. Weissmann (1999)
J. Biol. Chem. 274, 3-6
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Protease-resistant prion protein produced in vitro lacks detectable infectivity.
A. Hill, M Antoniou, and J Collinge (1999)
J. Gen. Virol. 80, 11-14
   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.
D. Westaway, G. Telling, and S. Priola (1998)
PNAS 95, 11030-11031
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A Transmembrane Form of the Prion Protein in Neurodegenerative Disease.
R. S. Hegde, J. A. Mastrianni, M. R. Scott, K. A. DeFea, P. Tremblay, M. Torchia, S. J. DeArmond, S. B. Prusiner, and V. R. Lingappa (1998)
Science 279, 827-834
   Abstract »    Full Text »
Identification of a prion protein epitope modulating transmission of bovine spongiform encephalopathy prions to transgenic mice.
M. R. Scott, J. Safar, G. Telling, O. Nguyen, D. Groth, M. Torchia, R. Koehler, P. Tremblay, D. Walther, F. E. Cohen, et al. (1997)
PNAS 94, 14279-14284
   Abstract »    Full Text »    PDF »
Prion Diseases and the BSE Crisis.
S. B. Prusiner (1997)
Science 278, 245-251
   Abstract »    Full Text »
Evidence for protein X binding to a discontinuous epitope on the cellular prion protein during scrapie prion propagation.
K. Kaneko, L. Zulianello, M. Scott, C. M. Cooper, A. C. Wallace, T. L. James, F. E. Cohen, and S. B. Prusiner (1997)
PNAS 94, 10069-10074
   Abstract »    Full Text »    PDF »
Solution structure of a 142-residue recombinant prion protein corresponding to the infectious fragment of the scrapie isoform.
T. L. James, H. Liu, N. B. Ulyanov, S. Farr-Jones, H. Zhang, D. G. Donne, K. Kaneko, D. Groth, I. Mehlhorn, S. B. Prusiner, et al. (1997)
PNAS 94, 10086-10091
   Abstract »    Full Text »    PDF »
Evolution of a Strain of CJD That Induces BSE-Like Plaques.
L. Manuelidis, W. Fritch, and Y. Xi (1997)
Science 277, 94-98
   Abstract »    Full Text »
Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms.
A. Bossers, P. B. G. M. Belt, G. J. Raymond, B. Caughey, R. de Vries, and M. A. Smits (1997)
PNAS 94, 4931-4936
   Abstract »    Full Text »    PDF »


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