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In fatal familial insomnia (FFI), the protease-resistant fragment of PrP\n            <jats:sup>Sc<\/jats:sup>\n            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 PrP\n            <jats:sup>Sc<\/jats:sup>\n            fragment, whereas extracts from the brains of familial and sporadic Creutzfeldt-Jakob disease patients produced the 21-kilodalton PrP\n            <jats:sup>Sc<\/jats:sup>\n            fragment in these mice. The results presented indicate that the conformation of PrP\n            <jats:sup>Sc<\/jats:sup>\n            functions as a template in directing the formation of nascent PrP\n            <jats:sup>Sc<\/jats:sup>\n            and suggest a mechanism to explain strains of prions where diversity is encrypted in the conformation of PrP\n            <jats:sup>Sc<\/jats:sup>\n            .\n          <\/jats:p>","DOI":"10.1126\/science.274.5295.2079","type":"journal-article","created":{"date-parts":[[2002,7,27]],"date-time":"2002-07-27T09:35:26Z","timestamp":1027762526000},"page":"2079-2082","source":"Crossref","is-referenced-by-count":772,"title":["Evidence for the Conformation of the Pathologic Isoform of the Prion Protein Enciphering and Propagating Prion Diversity"],"prefix":"10.1126","volume":"274","author":[{"given":"Glenn C.","family":"Telling","sequence":"first","affiliation":[{"name":"G. C. Telling and J. 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