{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,2,21]],"date-time":"2025-02-21T03:04:37Z","timestamp":1740107077607,"version":"3.37.3"},"reference-count":21,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2024,11,20]],"date-time":"2024-11-20T00:00:00Z","timestamp":1732060800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"},{"start":{"date-parts":[[2024,11,20]],"date-time":"2024-11-20T00:00:00Z","timestamp":1732060800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/www.springernature.com\/gp\/researchers\/text-and-data-mining"}],"funder":[{"DOI":"10.13039\/501100003407","name":"Ministero dell\u2019Istruzione, dell\u2019Universit\u00e0 e della Ricerca","doi-asserted-by":"publisher","award":["MNESYS (PE0000006)","MNESYS (PE0000006)"],"award-info":[{"award-number":["MNESYS (PE0000006)","MNESYS (PE0000006)"]}],"id":[{"id":"10.13039\/501100003407","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003478","name":"Ministry of Health, Labour and Welfare","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003478","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"DOI":"10.1007\/s00401-024-02825-8","type":"journal-article","created":{"date-parts":[[2024,11,20]],"date-time":"2024-11-20T05:18:52Z","timestamp":1732079932000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Unsuccessful transmissions of atypical genetic Creutzfeldt\u2013Jakob disease (PRNP p.T183A-129M) in transgenic mice"],"prefix":"10.1007","volume":"148","author":[{"given":"Simone","family":"Baiardi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Claudia Marina","family":"Vargiu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shirou","family":"Mohri","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Otto","family":"Windl","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jochen","family":"Herms","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sabina","family":"Capellari","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuyuki","family":"Kitamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Piero","family":"Parchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2024,11,20]]},"reference":[{"key":"2825_CR1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1009123","volume":"17","author":"P Aguilar-Calvo","year":"2021","unstructured":"Aguilar-Calvo P, Callender JA, Sigurdson CJ (2021) Short and sweet: How glycans impact prion conversion, cofactor interactions, and cross-species transmission. PLoS Pathog 17:e1009123. https:\/\/doi.org\/10.1371\/journal.ppat.1009123","journal-title":"PLoS Pathog"},{"key":"2825_CR2","doi-asserted-by":"publisher","first-page":"707","DOI":"10.1007\/s00401-021-02350-y","volume":"142","author":"S Baiardi","year":"2021","unstructured":"Baiardi S, Rossi M, Mammana A, Appleby BS, Barria MA, Cal\u00ec I et al (2021) Phenotypic diversity of genetic Creutzfeldt-Jakob disease: a histo-molecular-based classification. Acta Neuropathol 142:707\u2013728. https:\/\/doi.org\/10.1007\/s00401-021-02350-y","journal-title":"Acta Neuropathol"},{"key":"2825_CR3","doi-asserted-by":"publisher","first-page":"723","DOI":"10.3390\/v10120723","volume":"10","author":"IV Baskakov","year":"2018","unstructured":"Baskakov IV, Katorcha E, Makarava N (2018) Prion Strain-Specific Structure and Pathology: A View from the Perspective of Glycobiology. Viruses 10:723. https:\/\/doi.org\/10.3390\/v10120723","journal-title":"Viruses"},{"key":"2825_CR4","doi-asserted-by":"publisher","first-page":"12005","DOI":"10.1073\/pnas.1004688107","volume":"107","author":"MT Bishop","year":"2010","unstructured":"Bishop MT, Will RG, Manson JC (2010) Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 107:12005\u201312010. https:\/\/doi.org\/10.1073\/pnas.1004688107","journal-title":"Proc Natl Acad Sci USA"},{"key":"2825_CR5","doi-asserted-by":"publisher","first-page":"27","DOI":"10.3233\/jad-2000-2104","volume":"2","author":"S Capellari","year":"2000","unstructured":"Capellari S, Zaidi SI, Long AC, Kwon EE, Petersen RB (2000) The Thr183Ala mutation, not the loss of the first glycosylation site, alters the physical properties of the prion protein. J Alzheimers Dis 2:27\u201335. https:\/\/doi.org\/10.3233\/jad-2000-2104","journal-title":"J Alzheimers Dis"},{"key":"2825_CR6","doi-asserted-by":"publisher","first-page":"1539","DOI":"10.1093\/brain\/awad399","volume":"147","author":"L Cracco","year":"2024","unstructured":"Cracco L, Cali I, Cohen ML, Aslam R, Notari S, Kong Q et al (2024) Efficient transmission of human prion diseases to a glycan-free prion protein-expressing host. Brain 147:1539\u20131552. https:\/\/doi.org\/10.1093\/brain\/awad399","journal-title":"Brain"},{"key":"2825_CR7","doi-asserted-by":"publisher","first-page":"110","DOI":"10.1086\/302702","volume":"66","author":"U Finckh","year":"2000","unstructured":"Finckh U, M\u00fcller-Thomsen T, Mann U, Eggers C, Marksteiner J, Meins W et al (2000) High prevalence of pathogenic mutations in patients with early-onset dementia detected by sequence analyses of four different genes. Am J Hum Genet 66:110\u2013117. https:\/\/doi.org\/10.1086\/302702","journal-title":"Am J Hum Genet"},{"key":"2825_CR8","doi-asserted-by":"publisher","first-page":"476","DOI":"10.1007\/s00401-004-0913-4","volume":"108","author":"E Grasbon-Frodl","year":"2004","unstructured":"Grasbon-Frodl E, Lorenz H, Mann U, Nitsch RM, Windl O, Kretzschmar HA (2004) Loss of glycosylation associated with the T183A mutation in human prion disease. Acta Neuropathol 108:476\u2013484. https:\/\/doi.org\/10.1007\/s00401-004-0913-4","journal-title":"Acta Neuropathol"},{"key":"2825_CR9","doi-asserted-by":"publisher","first-page":"10867","DOI":"10.1128\/JVI.01383-16","volume":"90","author":"E Jaumain","year":"2016","unstructured":"Jaumain E, Quadrio I, Herzog L, Reine F, Rezaei H, Andr\u00e9oletti O et al (2016) Absence of evidence for a causal link between bovine spongiform encephalopathy strain variant L-BSE and known forms of sporadic Creutzfeldt-Jakob disease in human PrP transgenic mice. J Virol 90:10867\u201310874. https:\/\/doi.org\/10.1128\/JVI.01383-16","journal-title":"J Virol"},{"key":"2825_CR10","doi-asserted-by":"publisher","first-page":"280","DOI":"10.1016\/S0006-291X(02)00476-X","volume":"294","author":"T Kitamoto","year":"2002","unstructured":"Kitamoto T, Mohri S, Ironside JW, Miyoshi I, Tanaka T, Kitamoto N et al (2002) Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions. Biochem Biophys Res Commun 294:280\u2013286. https:\/\/doi.org\/10.1016\/S0006-291X(02)00476-X","journal-title":"Biochem Biophys Res Commun"},{"key":"2825_CR11","doi-asserted-by":"publisher","first-page":"74","DOI":"10.1186\/2051-5960-1-74","volume":"1","author":"A Kobayashi","year":"2013","unstructured":"Kobayashi A, Iwasaki Y, Otsuka H, Yamada M, Yoshida M, Matsuura Y et al (2013) Deciphering the pathogenesis of sporadic Creutzfeldt-Jakob disease with codon 129 M\/V and type 2 abnormal prion protein. Acta Neuropathol Commun 1:74. https:\/\/doi.org\/10.1186\/2051-5960-1-74","journal-title":"Acta Neuropathol Commun"},{"key":"2825_CR12","doi-asserted-by":"publisher","first-page":"159","DOI":"10.1007\/s00401-015-1447-7","volume":"130","author":"A Kobayashi","year":"2015","unstructured":"Kobayashi A, Teruya K, Matsuura Y, Shirai T, Nakamura Y, Yamada M et al (2015) The influence of PRNP polymorphisms on human prion disease susceptibility: an update. Acta Neuropathol 130:159\u2013170. https:\/\/doi.org\/10.1007\/s00401-015-1447-7","journal-title":"Acta Neuropathol"},{"key":"2825_CR13","doi-asserted-by":"publisher","first-page":"1276","DOI":"10.1016\/j.ajpath.2019.02.012","volume":"189","author":"A Kobayashi","year":"2019","unstructured":"Kobayashi A, Iwasaki Y, Takao M, Saito Y, Iwaki T, Qi Z et al (2019) A novel combination of prion strain co-occurrence in patients with sporadic Creutzfeldt-Jakob disease. Am J Pathol 189:1276\u20131283. https:\/\/doi.org\/10.1016\/j.ajpath.2019.02.012","journal-title":"Am J Pathol"},{"key":"2825_CR14","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1111\/bpa.12638","volume":"29","author":"A Kobayashi","year":"2019","unstructured":"Kobayashi A, Matsuura Y, Takeuchi A, Yamada M, Miyoshi I, Mohri S et al (2019) A domain responsible for spontaneous conversion of bank vole prion protein. Brain Pathol 29:155\u2013163. https:\/\/doi.org\/10.1111\/bpa.12638","journal-title":"Brain Pathol"},{"key":"2825_CR15","doi-asserted-by":"publisher","first-page":"4784","DOI":"10.1073\/pnas.2627989100","volume":"100","author":"C Korth","year":"2003","unstructured":"Korth C, Kaneko K, Groth D, Heye N, Telling G, Mastrianni J et al (2003) Abbreviated incubation times for human prions in mice expressing a chimeric mouse-human prion protein transgene. Proc Natl Acad Sci USA 100:4784\u20134789. https:\/\/doi.org\/10.1073\/pnas.2627989100","journal-title":"Proc Natl Acad Sci USA"},{"key":"2825_CR16","doi-asserted-by":"publisher","first-page":"4540","DOI":"10.1016\/j.molcel.2021.08.011","volume":"81","author":"A Kraus","year":"2021","unstructured":"Kraus A, Hoyt F, Schwartz CL, Hansen B, Artikis E, Hughson AG et al (2021) High-resolution structure and strain comparison of infectious mammalian prions. Mol Cell 81:4540-4551.e6. https:\/\/doi.org\/10.1016\/j.molcel.2021.08.011","journal-title":"Mol Cell"},{"key":"2825_CR17","doi-asserted-by":"publisher","first-page":"219","DOI":"10.1016\/B978-0-444-63945-5.00013-1","volume":"153","author":"A Ladogana","year":"2018","unstructured":"Ladogana A, Kovacs GG (2018) Genetic Creutzfeldt-Jakob disease. Handb Clin Neurol 153:219\u2013242. https:\/\/doi.org\/10.1016\/B978-0-444-63945-5.00013-1","journal-title":"Handb Clin Neurol"},{"key":"2825_CR18","doi-asserted-by":"publisher","first-page":"138","DOI":"10.1002\/ana.410420203","volume":"42","author":"R Nitrini","year":"1997","unstructured":"Nitrini R, Rosemberg S, Passos-Bueno MR, da Silva LS, Iughetti P, Papadopoulos M et al (1997) Familial spongiform encephalopathy associated with a novel prion protein gene mutation. Ann Neurol 42:138\u2013146. https:\/\/doi.org\/10.1002\/ana.410420203","journal-title":"Ann Neurol"},{"key":"2825_CR19","doi-asserted-by":"publisher","first-page":"767","DOI":"10.1002\/ana.410390613","volume":"39","author":"P Parchi","year":"1996","unstructured":"Parchi P, Castellani R, Capellari S, Ghetti B, Young K, Chen SG et al (1996) Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 39:767\u2013778. https:\/\/doi.org\/10.1002\/ana.410390613","journal-title":"Ann Neurol"},{"key":"2825_CR20","doi-asserted-by":"publisher","DOI":"10.1073\/pnas.2019631118","volume":"118","author":"M Sanz-Hern\u00e1ndez","year":"2021","unstructured":"Sanz-Hern\u00e1ndez M, Barritt JD, Sobek J, Hornemann S, Aguzzi A, De Simone A (2021) Mechanism of misfolding of the human prion protein revealed by a pathological mutation. Proc Natl Acad Sci USA 118:e2019631118. https:\/\/doi.org\/10.1073\/pnas.2019631118","journal-title":"Proc Natl Acad Sci USA"},{"key":"2825_CR21","doi-asserted-by":"publisher","first-page":"2585","DOI":"10.1016\/S0002-9440(10)63613-9","volume":"163","author":"Y Taguchi","year":"2003","unstructured":"Taguchi Y, Mohri S, Ironside JW, Muramoto T, Kitamoto T (2003) Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol 163:2585\u20132593. https:\/\/doi.org\/10.1016\/S0002-9440(10)63613-9","journal-title":"Am J Pathol"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-024-02825-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s00401-024-02825-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-024-02825-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,11,20]],"date-time":"2024-11-20T08:33:40Z","timestamp":1732091620000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/10.1007\/s00401-024-02825-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2024,11,20]]},"references-count":21,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2024,12]]}},"alternative-id":["2825"],"URL":"https:\/\/doi.org\/10.1007\/s00401-024-02825-8","relation":{},"ISSN":["1432-0533"],"issn-type":[{"type":"electronic","value":"1432-0533"}],"subject":[],"published":{"date-parts":[[2024,11,20]]},"assertion":[{"value":"7 August 2024","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 November 2024","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"4 November 2024","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"20 November 2024","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The authors declare no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and\/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. Medical information was collected as part of national surveillance programs for prion diseases and approved by the local Institutional Review Board for each participating center.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethical approval"}}],"article-number":"67"}}