{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T23:07:07Z","timestamp":1791328027984,"version":"4.3.1"},"reference-count":0,"publisher":"National Academy of Sciences","issue":"21","content-domain":{"domain":["www.pnas.org"],"crossmark-restriction":true},"short-container-title":["Proc. Natl. Acad. Sci. U.S.A."],"published-print":{"date-parts":[[1994,10,11]]},"abstract":"<jats:p>Transgenic (Tg) mice were constructed that express a chimeric prion protein (PrP) in which a segment of mouse (Mo) PrP was replaced with the corresponding human (Hu) PrP sequence. The chimeric PrP, designated MHu2MPrP, differs from MoPrP by 9 amino acids between residues 96 and 167. All of the Tg(MHu2M) mice developed neurologic disease approximately 200 days after inoculation with brain homogenates from three patients dying of Creutzfeldt-Jakob disease (CJD). Inoculation of Tg(MHu2M) mice with CJD prions produced MHu2MPrPSc (where PrPSc is the scrapie isoform of PrP); inoculation with Mo prions produced Mo-PrPSc. The patterns of MHu2MPrPSc and MoPrPSc accumulation in the brains of Tg(MHu2M) mice were different. About 10% of Tg(HuPrP) mice expressing HuPrP and non-Tg mice developed neurologic disease &gt; 500 days after inoculation with CJD prions. The different susceptibilities of Tg(HuPrP) and Tg(MHu2M) mice to Hu prions indicate that additional species-specific factors are involved in prion replication. Diagnosis, prevention, and treatment of Hu prion diseases should be facilitated by Tg(MHu2M) mice.<\/jats:p>","DOI":"10.1073\/pnas.91.21.9936","type":"journal-article","created":{"date-parts":[[2006,5,31]],"date-time":"2006-05-31T08:57:05Z","timestamp":1149065825000},"page":"9936-9940","update-policy":"https:\/\/doi.org\/10.1073\/pnas.cm10313","source":"Crossref","is-referenced-by-count":258,"title":["Transmission of Creutzfeldt-Jakob disease from humans to transgenic mice expressing chimeric human-mouse prion protein."],"prefix":"10.1073","volume":"91","author":[{"given":"G C","family":"Telling","sequence":"first","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Scott","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K K","family":"Hsiao","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D","family":"Foster","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S L","family":"Yang","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M","family":"Torchia","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K C","family":"Sidle","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J","family":"Collinge","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S J","family":"DeArmond","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S B","family":"Prusiner","sequence":"additional","affiliation":[{"name":"Department of Neurology, University of California, San Francisco 94143."}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"341","published-online":{"date-parts":[[1994,10,11]]},"container-title":["Proceedings of the National Academy of Sciences"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/pnas.org\/doi\/pdf\/10.1073\/pnas.91.21.9936","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,4,13]],"date-time":"2022-04-13T13:52:39Z","timestamp":1649857959000},"score":1,"resource":{"primary":{"URL":"https:\/\/pnas.org\/doi\/full\/10.1073\/pnas.91.21.9936"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[1994,10,11]]},"references-count":0,"journal-issue":{"issue":"21","published-print":{"date-parts":[[1994,10,11]]}},"alternative-id":["10.1073\/pnas.91.21.9936"],"URL":"https:\/\/doi.org\/10.1073\/pnas.91.21.9936","relation":{},"ISSN":["0027-8424","1091-6490"],"issn-type":[{"value":"0027-8424","type":"print"},{"value":"1091-6490","type":"electronic"}],"subject":[],"published":{"date-parts":[[1994,10,11]]},"assertion":[{"value":"1994-10-11","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}