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Prions are transmissible particles that are devoid of nucleic acid and seem to be composed exclusively of a modified protein (PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    ). The normal, cellular PrP (PrP\n                    <jats:sup>C<\/jats:sup>\n                    ) is converted into PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    through a posttranslational process during which it acquires a high \u03b2-sheet content. The species of a particular prion is encoded by the sequence of the chromosomal PrP gene of the mammals in which it last replicated. In contrast to pathogens carrying a nucleic acid genome, prions appear to encipher strain-specific properties in the tertiary structure of PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    . Transgenetic studies argue that PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    acts as a template upon which PrP\n                    <jats:sup>C<\/jats:sup>\n                    is refolded into a nascent PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    molecule through a process facilitated by another protein. Miniprions generated in transgenic mice expressing PrP, in which nearly half of the residues were deleted, exhibit unique biological properties and should facilitate structural studies of PrP\n                    <jats:sup>Sc<\/jats:sup>\n                    . While knowledge about prions has profound implications for studies of the structural plasticity of proteins, investigations of prion diseases suggest that new strategies for the prevention and treatment of these disorders may also find application in the more common degenerative diseases.\n                  <\/jats:p>","DOI":"10.1073\/pnas.95.23.13363","type":"journal-article","created":{"date-parts":[[2002,7,26]],"date-time":"2002-07-26T10:42:40Z","timestamp":1027680160000},"page":"13363-13383","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":5007,"title":["Prions"],"prefix":"10.1073","volume":"95","author":[{"given":"Stanley B.","family":"Prusiner","sequence":"first","affiliation":[{"name":"Departments of Neurology and of Biochemistry and Biophysics, University of California, San Francisco, CA 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