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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.
Read the Full Text
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96, 15137-15142
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- Glycosylation differences between the normal and pathogenic prion protein isoforms.
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PNAS
96, 13044-13049
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- Type 1 protease resistant prion protein and valine homozygosity at codon 129 of PRNP identify a subtype of sporadic Creutzfeldt-Jakob disease.
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J. Neurol. Neurosurg. Psychiatry
67, 671-674
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- Protease-resistant and Detergent-insoluble Prion Protein Is Not Necessarily Associated with Prion Infectivity.
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274, 17981-17986
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- Prion Protein Conformation in a Patient with Sporadic Fatal Insomnia.
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N. Engl. J. Med.
340, 1630-1638
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- Molecular Genetics of Transmissible Spongiform Encephalopathies.
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J. Biol. Chem.
274, 3-6
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- Protease-resistant prion protein produced in vitro lacks detectable infectivity.
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J. Gen. Virol.
80, 11-14
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- Strain-dependent Differences in beta -Sheet Conformations of Abnormal Prion Protein.
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273, 32230-32235
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PNAS
95, 13363-13383
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