{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,3]],"date-time":"2026-10-03T17:07:55Z","timestamp":1791047275427,"version":"4.1.0"},"reference-count":25,"publisher":"National Academy of Sciences","issue":"6","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[2005,2,8]]},"abstract":"<jats:p>\n                    Synthetic prions were produced in our laboratory by using recombinant mouse prion protein (MoPrP) composed of residues 89-230. The first mouse synthetic prion strain (MoSP1) was inoculated into transgenic (Tg) 9949 mice expressing N-terminally truncated MoPrP(\u039423-88) and WT FVB mice expressing full-length MoPrP. On first and second passage in Tg9949 mice, MoSP1 prions caused disease in 516 \u00b1 27 and 258 \u00b1 25 days, respectively; numerous, large vacuoles were found in the brainstem and gray matter of the cerebellum. MoSP1 prions passaged in Tg9949 mice were inoculated into FVB mice; on first and second passage, the FVB mice exhibited incubation times of 154 \u00b1 4 and 130 \u00b1 3 days, respectively. In FVB mice, vacuolation was less intense but more widely distributed, with numerous lesions in the hippocampus and cerebellar white matter. This constellation of widespread neuropatho-logic changes was similar to that found in FVB mice inoculated with Rocky Mountain Laboratory (RML) prions, a strain derived from a sheep with scrapie. Conformational stability studies showed that the half-maximal GdnHCl (Gdn\n                    <jats:sub>1\/2<\/jats:sub>\n                    ) concentration for denaturation of MoSP1 prions passaged in Tg9949 mice was \u22484.2 M; passage in FVB mice reduced the Gdn\n                    <jats:sub>1\/2<\/jats:sub>\n                    value to \u22481.7 M. RML prions passaged in either Tg9949 or FVB mice exhibited Gdn\n                    <jats:sub>1\/2<\/jats:sub>\n                    values of \u22481.8 M. The incubation times, neuropathological lesion profiles, and Gdn\n                    <jats:sub>1\/2<\/jats:sub>\n                    values indicate that MoSP1 prions differ from RML and many other prion strains derived from sheep with scrapie and cattle with bovine spongiform encephalopathy.\n                  <\/jats:p>","DOI":"10.1073\/pnas.0409079102","type":"journal-article","created":{"date-parts":[[2005,1,25]],"date-time":"2005-01-25T22:43:38Z","timestamp":1106693018000},"page":"2168-2173","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":166,"title":["Strain-specified characteristics of mouse synthetic prions"],"prefix":"10.1073","volume":"102","author":[{"given":"Giuseppe","family":"Legname","sequence":"first","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hoang-Oanh B.","family":"Nguyen","sequence":"additional","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ilia V.","family":"Baskakov","sequence":"additional","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fred E.","family":"Cohen","sequence":"additional","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen J.","family":"DeArmond","sequence":"additional","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stanley B.","family":"Prusiner","sequence":"additional","affiliation":[{"name":"Institute for Neurodegenerative Diseases and Departments of Neurology, Cellular and Molecular Pharmacology, Biochemistry and Biophysics, and Pathology, University of California, San Francisco, CA 94143"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[2005,1,25]]},"reference":[{"key":"e_1_3_2_1_2","unstructured":"Prusiner S. 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