{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T04:46:44Z","timestamp":1790398004731,"version":"4.1.0"},"reference-count":32,"publisher":"Springer Science and Business Media LLC","issue":"1","funder":[{"DOI":"10.13039\/501100003196","name":"Ministero della Salute","doi-asserted-by":"publisher","award":["RF-2009-1474624"],"award-info":[{"award-number":["RF-2009-1474624"]}],"id":[{"id":"10.13039\/501100003196","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100005969","name":"Universit\u00e0 di Bologna","doi-asserted-by":"publisher","award":["RFO 2013-16"],"award-info":[{"award-number":["RFO 2013-16"]}],"id":[{"id":"10.13039\/501100005969","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Fondazione Galletti"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1186\/s40478-017-0496-7","type":"journal-article","created":{"date-parts":[[2017,11,23]],"date-time":"2017-11-23T07:17:57Z","timestamp":1511421477000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":31,"title":["Atypical Creutzfeldt-Jakob disease with PrP-amyloid plaques in white matter: molecular characterization and transmission to bank voles show the M1 strain signature"],"prefix":"10.1186","volume":"5","author":[{"given":"Marcello","family":"Rossi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniela","family":"Saverioni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele","family":"Di Bari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simone","family":"Baiardi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Afina Willemina","family":"Lemstra","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Laura","family":"Pirisinu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sabina","family":"Capellari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annemieke","family":"Rozemuller","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romolo","family":"Nonno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9444-9524","authenticated-orcid":false,"given":"Piero","family":"Parchi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2017,11,23]]},"reference":[{"issue":"4","key":"496_CR1","doi-asserted-by":"publisher","first-page":"336","DOI":"10.1111\/neup.12192","volume":"35","author":"AS Berghoff","year":"2015","unstructured":"Berghoff AS, Trummert A, Unterberger U, Str\u00f6bel T, Hortob\u00e1gyi T, Kovacs GG (2015) Atypical sporadic CJD-MM phenotype with white matter kuru plaques associated with intranuclear inclusion body and argyrophilic grain disease. Neuropathology 35(4):336\u2013342. doi:\n10.1111\/neup.12192","journal-title":"Neuropathology"},{"key":"496_CR2","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 U S A 107:12005\u201312010. doi:\n10.1073\/pnas.1004688107","journal-title":"Proc Natl Acad Sci U S A"},{"key":"496_CR3","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1038\/39057","volume":"389","author":"ME Bruce","year":"1997","unstructured":"Bruce ME, Will RG, Ironside JW, McConnell I, Drummond D, Suttie A et al (1997) Transmissions to mice indicate that \u201cnew variant\u201d CJD is caused by the BSE agent. Nature 389:498\u2013501. doi:\n10.1038\/39057","journal-title":"Nature"},{"key":"496_CR4","first-page":"35","volume":"207","author":"GA Carlson","year":"1996","unstructured":"Carlson GA (1996) Prion strains. Curr Top Microbiol Immunol 207:35\u201347","journal-title":"Curr Top Microbiol Immunol"},{"key":"496_CR5","doi-asserted-by":"crossref","unstructured":"Cartoni C, Schinin\u00e0 ME, Maras B, Nonno R, Vaccari G, Di Baria MA et al. (2005) Identification of the pathological prion protein allotypes in scrapie-infected heterozygous bank voles (Clethrionomys glareolus) by high-performance liquid chromatography-mass spectrometry. J Chromatogr A 1081:122\u2013126","DOI":"10.1016\/j.chroma.2005.04.035"},{"key":"496_CR6","doi-asserted-by":"publisher","first-page":"6244","DOI":"10.1128\/JVI.00144-16","volume":"90","author":"M Cescatti","year":"2016","unstructured":"Cescatti M, Saverioni D, Capellari S, Tagliavini F, Kitamoto T, Ironside J et al (2016) Analysis of conformational stability of abnormal prion protein aggregates across the Spectrum of Creutzfeldt-Jakob disease prions. J Virol 90:6244\u20136254. doi:\n10.1128\/JVI.00144-16","journal-title":"J Virol"},{"key":"496_CR7","doi-asserted-by":"publisher","first-page":"453","DOI":"10.1007\/978-1-61779-551-0_31","volume":"849","author":"MA Di Bari","year":"2012","unstructured":"Di Bari MA, Nonno R, Agrimi U (2012) The mouse model for scrapie: inoculation, clinical scoring, and histopathological techniques. Methods Mol Biol 849:453\u2013471. doi:\n10.1007\/978-1-61779-551-0_31","journal-title":"Methods Mol Biol"},{"key":"496_CR8","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1003219","volume":"9","author":"MA Di Bari","year":"2013","unstructured":"Di Bari MA, Nonno R, Castilla J, D\u2019Agostino C, Pirisinu L, al RG (2013) Chronic wasting disease in bank voles: characterisation of the shortest incubation time model for prion diseases. PLoS Pathog 9:e1003219. doi:\n10.1371\/journal.ppat.1003219","journal-title":"PLoS Pathog"},{"key":"496_CR9","doi-asserted-by":"publisher","first-page":"204","DOI":"10.1111\/j.1440-1789.2012.01341.x","volume":"33","author":"E Gelpi","year":"2013","unstructured":"Gelpi E, Soler Insa JM, Parchi P, Saverioni D, Yag\u00fce J, Nos C et al (2013) Atypical neuropathological sCJD-MM phenotype with abundant white matter Kuru-type plaques sparing the cerebellar cortex. Neuropathology 33:204\u2013208. doi:\n10.1111\/j.1440-1789.2012.01341.x","journal-title":"Neuropathology"},{"key":"496_CR10","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0036333","volume":"7","author":"C Jansen","year":"2012","unstructured":"Jansen C, Parchi P, Capellari S, Ibrahim-Verbaas CA, Schuur M, Strammiello R et al (2012) Human prion diseases in the Netherlands (1998-2009): clinical, genetic and molecular aspects. PLoS One 7:e36333. doi:\n10.1371\/journal.pone.0036333","journal-title":"PLoS One"},{"key":"496_CR11","first-page":"1285","volume":"140","author":"T Kitamoto","year":"1992","unstructured":"Kitamoto T, Shin RW, Doh-ura K, Tomokane N, Miyazono M, Muramoto T et al (1992) Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease. Am J Pathol 140:1285\u20131294","journal-title":"Am J Pathol"},{"key":"496_CR12","doi-asserted-by":"publisher","first-page":"561","DOI":"10.1007\/s00401-008-0425-8","volume":"116","author":"A Kobayashi","year":"2008","unstructured":"Kobayashi A, Arima K, Ogawa M, Murata M, Fukuda T, Kitamoto T (2008) Plaque-type deposition of prion protein in the damaged white matter of sporadic Creutzfeldt-Jakob disease MM1 patients. Acta Neuropathol 116:561\u2013566. doi:\n10.1007\/s00401-008-0425-8","journal-title":"Acta Neuropathol"},{"key":"496_CR13","doi-asserted-by":"publisher","DOI":"10.1186\/2051-5960-2-32","volume":"2","author":"A Kobayashi","year":"2014","unstructured":"Kobayashi A, Matsuura Y, Mohri S, Kitamoto T (2014) Distinct origins of dura mater graft-associated Creutzfeldt-Jakob disease: past and future problems. Acta Neuropathol Commun 2:32. doi:\n10.1186\/2051-5960-2-32","journal-title":"Acta Neuropathol Commun"},{"key":"496_CR14","doi-asserted-by":"publisher","first-page":"3939","DOI":"10.1128\/JVI.03183-14","volume":"89","author":"A Kobayashi","year":"2015","unstructured":"Kobayashi A, Parchi P, Yamada M, Brown P, Saverioni D, Matsuura Y et al (2015) Transmission properties of atypical Creutzfeldt-Jakob disease: a clue to disease etiology? J Virol 89:3939\u20133946. doi:\n10.1128\/JVI.03183-14","journal-title":"J Virol"},{"key":"496_CR15","doi-asserted-by":"publisher","first-page":"3230","DOI":"10.1128\/JVI.02387-09","volume":"84","author":"A Kobayashi","year":"2010","unstructured":"Kobayashi A, Sakuma N, Matsuura Y, Mohri S, Aguzzi A, Kitamoto T (2010) Experimental verification of a traceback phenomenon in prion infection. J Virol 84:3230\u20133238. doi:\n10.1128\/JVI.02387-09","journal-title":"J Virol"},{"key":"496_CR16","doi-asserted-by":"publisher","first-page":"559","DOI":"10.1007\/s00401-017-1683-0","volume":"133","author":"F Lattanzio","year":"2017","unstructured":"Lattanzio F, Abu-Rumeileh S, Franceschini A, Kai H, Amore G, Poggiolini I et al (2017) Prion-specific and surrogate CSF biomarkers in Creutzfeldt-Jakob disease: diagnostic accuracy in relation to molecular subtypes and analysis of neuropathological correlates of p-tau and A\u03b242 levels. Acta Neuropathol 133:559\u2013578. doi:\n10.1007\/s00401-017-1683-0","journal-title":"Acta Neuropathol"},{"key":"496_CR17","doi-asserted-by":"publisher","first-page":"662","DOI":"10.1111\/j.1750-3639.2012.00572.x","volume":"22","author":"F Moda","year":"2012","unstructured":"Moda F, Suardi S, Di Fede G, Indaco A, Limido L, Vimercati C et al (2012) MM2-thalamic Creutzfeldt-Jakob disease: neuropathological, biochemical and transmission studies identify a distinctive prion strain. Brain Pathol 22:662\u2013669. doi:\n10.1111\/j.1750-3639.2012.00572.x","journal-title":"Brain Pathol"},{"key":"496_CR18","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.0020012","volume":"2","author":"R Nonno","year":"2006","unstructured":"Nonno R, Di Bari MA, Cardone F, Vaccari G, Fazzi P, Dell\u2019Omo G et al (2006) Efficient transmission and characterization of Creutzfeldt-Jakob disease strains in bank voles. PLoS Pathog 2:e12. doi:\n10.1371\/journal.ppat.0020012","journal-title":"PLoS Pathog"},{"key":"496_CR19","doi-asserted-by":"publisher","first-page":"16797","DOI":"10.1074\/jbc.M313220200","volume":"279","author":"S Notari","year":"2004","unstructured":"Notari S, Capellari S, Giese A, Westner I, Baruzzi A, Ghetti B et al (2004) Effects of different experimental conditions on the PrPSc core generated by protease digestion: implications for strain typing and molecular classification of CJD. J Biol Chem 279:16797\u201316804. doi:\n10.1074\/jbc.M313220200","journal-title":"J Biol Chem"},{"key":"496_CR20","doi-asserted-by":"publisher","first-page":"30557","DOI":"10.1074\/jbc.M801877200","volume":"283","author":"S Notari","year":"2008","unstructured":"Notari S, Strammiello R, Capellari S, Giese A, Cescatti M, Grassi J et al (2008) Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem 283:30557\u201330565. doi:\n10.1074\/jbc.M801877200","journal-title":"J Biol Chem"},{"key":"496_CR21","doi-asserted-by":"publisher","first-page":"517","DOI":"10.1007\/s00401-012-1002-8","volume":"124","author":"P Parchi","year":"2012","unstructured":"Parchi P, de Boni L, Saverioni D, Cohen ML, Ferrer I, Gambetti P et al (2012) Consensus classification of human prion disease histotypes allows reliable identification of molecular subtypes: an inter-rater study among surveillance centres in Europe and USA. Acta Neuropathol 124:517\u2013529. doi:\n10.1007\/s00401-012-1002-8","journal-title":"Acta Neuropathol"},{"key":"496_CR22","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. doi:\n10.1002\/ana.410390613","journal-title":"Ann Neurol"},{"key":"496_CR23","doi-asserted-by":"publisher","first-page":"3030","DOI":"10.1093\/brain\/awq234","volume":"133","author":"P Parchi","year":"2010","unstructured":"Parchi P, Cescatti M, Notari S, Schulz-Schaeffer WJ, Capellari S, Giese A et al (2010) Agent strain variation in human prion disease: insights from a molecular and pathological review of the National Institutes of Health series of experimentally transmitted disease. Brain 133:3030\u20133042. doi:\n10.1093\/brain\/awq234","journal-title":"Brain"},{"key":"496_CR24","doi-asserted-by":"crossref","first-page":"224","DOI":"10.1002\/1531-8249(199908)46:2<224::AID-ANA12>3.0.CO;2-W","volume":"46","author":"P Parchi","year":"1999","unstructured":"Parchi P, Giese A, Capellari S, Brown P, Schulz-Schaeffer W, Windl O et al (1999) Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224\u2013233","journal-title":"Ann Neurol"},{"key":"496_CR25","doi-asserted-by":"publisher","first-page":"659","DOI":"10.1007\/s00401-009-0585-1","volume":"118","author":"P Parchi","year":"2009","unstructured":"Parchi P, Strammiello R, Notari S, Giese A, Langeveld JPM, Ladogana A et al (2009) Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrPSc types: an updated classification. Acta Neuropathol 118:659\u2013671. doi:\n10.1007\/s00401-009-0585-1","journal-title":"Acta Neuropathol"},{"key":"496_CR26","doi-asserted-by":"publisher","first-page":"446","DOI":"10.3390\/pathogens2030446","volume":"2","author":"L Pirisinu","year":"2013","unstructured":"Pirisinu L, Marcon S, Di Bari MA, D\u2019Agostino C, Agrimi U, Nonno R (2013) Biochemical characterization of prion strains in bank voles. Pathogens 2:446\u2013456. doi:\n10.3390\/pathogens2030446","journal-title":"Pathogens"},{"key":"496_CR27","doi-asserted-by":"crossref","first-page":"13363","DOI":"10.1073\/pnas.95.23.13363","volume":"95","author":"SB Prusiner","year":"1998","unstructured":"Prusiner SB (1998) Prions. Proc Natl Acad Sci U S A 95:13363\u201313383","journal-title":"Proc Natl Acad Sci U S A"},{"key":"496_CR28","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1007\/s00401-016-1638-x","volume":"133","author":"DL Ritchie","year":"2017","unstructured":"Ritchie DL, Barria MA, Peden AH, Yull HM, Kirkpatrick J, Adlard P et al (2017) UK iatrogenic Creutzfeldt-Jakob disease: investigating human prion transmission across genotypic barriers using human tissue-based and molecular approaches. Acta Neuropathol 133:579\u2013595. doi:\n10.1007\/s00401-016-1638-x","journal-title":"Acta Neuropathol"},{"key":"496_CR29","doi-asserted-by":"publisher","first-page":"27972","DOI":"10.1074\/jbc.M113.477547","volume":"288","author":"D Saverioni","year":"2013","unstructured":"Saverioni D, Notari S, Capellari S, Poggiolini I, Giese A, Kretzschmar HA et al (2013) Analyses of protease resistance and aggregation state of abnormal prion protein across the spectrum of human prions. J Biol Chem 288:27972\u201327985. doi:\n10.1074\/jbc.M113.477547","journal-title":"J Biol Chem"},{"key":"496_CR30","doi-asserted-by":"publisher","first-page":"2422","DOI":"10.1016\/j.bbamem.2015.07.014","volume":"1848","author":"Y Sun","year":"2015","unstructured":"Sun Y, Hung W-C, Lee M-T, Huang HW (2015) Membrane-mediated amyloid formation of PrP 106-126: a kinetic study. Biochim Biophys Acta 1848:2422\u20132429. \nhttps:\/\/doi.org\/10.1016\/j.bbamem.2015.07.014","journal-title":"Biochim Biophys Acta"},{"key":"496_CR31","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/S0140-6736(96)91412-9","volume":"347","author":"RG Will","year":"1996","unstructured":"Will RG, Ironside JW, Zeidler M, Cousens SN, Estibeiro K, Alperovitch A et al (1996) A new variant of Creutzfeldt-Jakob disease in the UK. Lancet 347:921\u2013925","journal-title":"Lancet"},{"key":"496_CR32","doi-asserted-by":"publisher","first-page":"40429","DOI":"10.1074\/jbc.M308550200","volume":"278","author":"W-Q Zou","year":"2003","unstructured":"Zou W-Q, Capellari S, Parchi P, Sy M-S, Gambetti P, Chen SG (2003) Identification of novel proteinase K-resistant C-terminal fragments of PrP in Creutzfeldt-Jakob disease. J Biol Chem 278:40429\u201340436. doi:\n10.1074\/jbc.M308550200","journal-title":"J Biol Chem"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-017-0496-7.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2017,11,23]],"date-time":"2017-11-23T07:18:12Z","timestamp":1511421492000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-017-0496-7"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,11,23]]},"references-count":32,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2017,12]]}},"alternative-id":["496"],"URL":"https:\/\/doi.org\/10.1186\/s40478-017-0496-7","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2017,11,23]]},"article-number":"87"}}