{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T04:46:43Z","timestamp":1790398003777,"version":"4.1.0"},"reference-count":37,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"published-print":{"date-parts":[[2013,12]]},"DOI":"10.1186\/2051-5960-1-74","type":"journal-article","created":{"date-parts":[[2013,11,13]],"date-time":"2013-11-13T12:02:18Z","timestamp":1384344138000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":41,"title":["Deciphering the pathogenesis of sporadic Creutzfeldt-Jakob disease with codon 129 M\/V and type 2 abnormal prion protein"],"prefix":"10.1186","volume":"1","author":[{"given":"Atsushi","family":"Kobayashi","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yasushi","family":"Iwasaki","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hiroyuki","family":"Otsuka","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Masahito","family":"Yamada","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mari","family":"Yoshida","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yuichi","family":"Matsuura","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shirou","family":"Mohri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Tetsuyuki","family":"Kitamoto","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2013,11,13]]},"reference":[{"key":"67_CR1","doi-asserted-by":"publisher","first-page":"337","DOI":"10.1016\/S0092-8674(00)81163-0","volume":"93","author":"SB Prusiner","year":"1998","unstructured":"Prusiner SB, Scott MR, DeArmond SJ, Cohen FE: Prion protein biology. Cell 1998, 93: 337\u2013348.","journal-title":"Cell"},{"key":"67_CR2","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.: Molecular basis of phenotypic variability in sporadic Creutzfeldt-Jakob disease. Ann Neurol 1996, 39: 767\u2013778.","journal-title":"Ann Neurol"},{"key":"67_CR3","doi-asserted-by":"publisher","first-page":"232","DOI":"10.1038\/386232a0","volume":"386","author":"P Parchi","year":"1997","unstructured":"Parchi P, Capellari S, Chen SG, Petersen RB, Gambetti P, Kopp N, et al.: Typing prion isoforms. Nature 1997, 386: 232\u2013234.","journal-title":"Nature"},{"key":"67_CR4","doi-asserted-by":"publisher","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.: Classification of sporadic Creutzfeldt-Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 1999, 46: 224\u2013233.","journal-title":"Ann Neurol"},{"key":"67_CR5","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1007\/s00401-010-0779-6","volume":"121","author":"P Parchi","year":"2011","unstructured":"Parchi P, Strammiello R, Giese A, Kretzschmar H: Phenotypic variability of sporadic human prion disease and its molecular basis: past, present, and future. Acta Neuropathol 2011, 121: 91\u2013112.","journal-title":"Acta Neuropathol"},{"key":"67_CR6","doi-asserted-by":"publisher","first-page":"10168","DOI":"10.1073\/pnas.97.18.10168","volume":"97","author":"P Parchi","year":"2000","unstructured":"Parchi P, Zou W, Wang W, Brown P, Capellari S, Ghetti B, et al.: Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci USA 2000, 97: 10168\u201310172.","journal-title":"Proc Natl Acad Sci USA"},{"key":"67_CR7","doi-asserted-by":"publisher","first-page":"685","DOI":"10.1038\/383685a0","volume":"383","author":"J Collinge","year":"1996","unstructured":"Collinge J, Sidle KC, Meads J, Ironside J, Hill AF: Molecular analysis of prion strain variation and the aetiology of \u2018new variant\u2019 CJD. Nature 1996, 383: 685\u2013690.","journal-title":"Nature"},{"key":"67_CR8","doi-asserted-by":"publisher","first-page":"1333","DOI":"10.1093\/brain\/awg125","volume":"126","author":"AF Hill","year":"2003","unstructured":"Hill AF, Joiner S, Wadsworth JD, Sidle KC, Bell JE, Budka H, et al.: Molecular classification of sporadic Creutzfeldt-Jakob disease. Brain 2003, 126: 1333\u20131346.","journal-title":"Brain"},{"key":"67_CR9","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 JP, Ladogana A, et al.: Incidence and spectrum of sporadic Creutzfeldt-Jakob disease variants with mixed phenotype and co-occurrence of PrP\n                    Sc\n                   types: an updated classification. Acta Neuropathol 2009, 118: 659\u2013671.","journal-title":"Acta Neuropathol"},{"key":"67_CR10","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.: 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 2012, 124: 517\u2013529.","journal-title":"Acta Neuropathol"},{"key":"67_CR11","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.: Effects of different experimental conditions on the PrP\n                    Sc\n                   core generated by protease digestion. J Biol Chem 2004, 279: 16797\u201316804.","journal-title":"J Biol Chem"},{"key":"67_CR12","doi-asserted-by":"publisher","first-page":"1103","DOI":"10.1038\/labinvest.3700676","volume":"87","author":"S Notari","year":"2007","unstructured":"Notari S, Capellari S, Langeveld J, Giese A, Strammiello R, Gambetti P, et al.: A refined method for molecular typing reveals that co-occurrence of PrP\n                    Sc\n                   types in Creutzfeldt-Jakob disease is not the rule. Lab Invest 2007, 87: 1103\u20131112.","journal-title":"Lab Invest"},{"key":"67_CR13","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: Defining sporadic Creutzfeldt-Jakob disease strains and their transmission properties. Proc Natl Acad Sci USA 2010, 107: 12005\u201312010.","journal-title":"Proc Natl Acad Sci USA"},{"key":"67_CR14","first-page":"1285","volume":"140","author":"T Kitamoto","year":"1992","unstructured":"Kitamoto T, Shin R-W, Doh-ura K, Tomokane N, Miyazono M, Muramoto T, et al.: Abnormal isoform of prion proteins accumulates in the synaptic structures of the central nervous system in patients with Creutzfeldt-Jakob disease. Am J Pathol 1992, 140: 1285\u20131294.","journal-title":"Am J Pathol"},{"key":"67_CR15","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: Humanized knock-in mice expressing chimeric prion protein showed varied susceptibility to different human prions. Am J Pathol 2003, 163: 2585\u20132593.","journal-title":"Am J Pathol"},{"key":"67_CR16","doi-asserted-by":"publisher","first-page":"709","DOI":"10.1006\/bbrc.1993.1275","volume":"191","author":"T Kitamoto","year":"1993","unstructured":"Kitamoto T, Ohta M, Doh-ura K, Hitoshi S, Terao Y, Tateishi J: Novel missense variants of prion protein in Creutzfeldt-Jakob disease or Gerstmann-Str\u00e4ussler syndrome. Biochem Biophys Res Commun 1993, 191: 709\u2013714.","journal-title":"Biochem Biophys Res Commun"},{"key":"67_CR17","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.: Follicular dendritic cell of the knock-in mouse provides a new bioassay for human prions. Biochem Biophys Res Commun 2002, 294: 280\u2013286.","journal-title":"Biochem Biophys Res Commun"},{"key":"67_CR18","doi-asserted-by":"publisher","first-page":"293","DOI":"10.1016\/j.bbrc.2006.01.149","volume":"342","author":"M Asano","year":"2006","unstructured":"Asano M, Mohri S, Ironside JW, Ito M, Tamaoki N, Kitamoto T: vCJD prion acquires altered virulence through trans-species infection. Biochem Biophys Res Commun 2006, 342: 293\u2013299.","journal-title":"Biochem Biophys Res Commun"},{"key":"67_CR19","doi-asserted-by":"crossref","first-page":"3688","DOI":"10.1128\/jvi.61.12.3688-3693.1987","volume":"61","author":"RJ Kascsak","year":"1987","unstructured":"Kascsak RJ, Rubenstein R, Merz PA, Tonna-DeMasi M, Fersko R, Carp RI, et al.: Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J Virol 1987, 61: 3688\u20133693.","journal-title":"J Virol"},{"key":"67_CR20","doi-asserted-by":"publisher","first-page":"179","DOI":"10.1016\/S0304-3940(00)01232-5","volume":"288","author":"T Muramoto","year":"2000","unstructured":"Muramoto T, Tanaka T, Kitamoto N, Sano C, Hayashi Y, Kutomi T, et al.: Analyses of Gerstmann-Straussler syndrome with 102Leu219Lys using monoclonal antibodies that specifically detect human prion protein with 219Glu. Neurosci Lett 2000, 288: 179\u2013182.","journal-title":"Neurosci Lett"},{"key":"67_CR21","doi-asserted-by":"publisher","first-page":"2885","DOI":"10.1099\/vir.0.19236-0","volume":"84","author":"K Satoh","year":"2003","unstructured":"Satoh K, Muramoto T, Tanaka T, Kitamoto N, Ironside JW, Nagashima K, et al.: Association of an 11\u201312 kDa protease-resistant prion protein fragment with subtypes of dura graft-associated Creutzfeldt-Jakob disease and other prion diseases. J Gen Virol 2003, 84: 2885\u20132893.","journal-title":"J Gen Virol"},{"key":"67_CR22","doi-asserted-by":"publisher","first-page":"319","DOI":"10.1016\/0006-8993(91)91306-L","volume":"545","author":"T Kitamoto","year":"1991","unstructured":"Kitamoto T, Muramoto T, Hilbich C, Beyreuther K, Tateishi J: N-terminal sequence of prion protein is also integrated into kuru plaques in patients with Gerstmann-Str\u00e4ussler syndrome. Brain Res 1991, 545: 319\u2013321.","journal-title":"Brain Res"},{"key":"67_CR23","doi-asserted-by":"publisher","first-page":"1863","DOI":"10.1007\/BF01718200","volume":"141","author":"KUD Grathwohl","year":"1996","unstructured":"Grathwohl KUD, Horiuchi M, Ishiguro N, Shinagawa M: Improvement of PrP\n                    Sc\n                  \n-detection in mouse spleen early at the preclinical stage of scrapie with collagenase-completed tissue homogenization and Sarkosyl-NaCl extraction of PrP\n                    Sc\n                  \n. Arch Virol 1996, 141: 1863\u20131874.","journal-title":"Arch Virol"},{"key":"67_CR24","doi-asserted-by":"publisher","first-page":"1195","DOI":"10.1016\/j.bbrc.2008.03.014","volume":"369","author":"S Ikeda","year":"2008","unstructured":"Ikeda S, Kobayashi A, Kitamoto T: Thr but Asn of the N-glycosylation sites of PrP is indispensable for its misfolding. Biochem Biophys Res Commun 2008, 369: 1195\u20131198.","journal-title":"Biochem Biophys Res Commun"},{"key":"67_CR25","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: Experimental verification of a traceback phenomenon in prion infection. J Virol 2010, 84: 3230\u20133238.","journal-title":"J Virol"},{"key":"67_CR26","doi-asserted-by":"publisher","first-page":"805","DOI":"10.1016\/S1474-4422(05)70225-8","volume":"4","author":"M Polymenidou","year":"2005","unstructured":"Polymenidou M, Stoeck K, Glatzel M, Vey M, Bellon A, Aguzzi A: Coexistence of multiple PrP\n                    Sc\n                   types in individuals with Creutzfeldt-Jakob disease. Lancet Neurol 2005, 4: 805\u2013814.","journal-title":"Lancet Neurol"},{"key":"67_CR27","doi-asserted-by":"publisher","first-page":"151","DOI":"10.2353\/ajpath.2006.050766","volume":"168","author":"HM Yull","year":"2006","unstructured":"Yull HM, Ritchie DL, Langeveld JP, van Zijderveld FG, Bruce ME, Ironside JW, et al.: Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol 2006, 168: 151\u2013157.","journal-title":"Am J Pathol"},{"key":"67_CR28","doi-asserted-by":"publisher","first-page":"1309","DOI":"10.1016\/j.ajpath.2010.11.069","volume":"178","author":"A Kobayashi","year":"2011","unstructured":"Kobayashi A, Mizukoshi K, Iwasaki Y, Miyata H, Yoshida Y, Kitamoto T: Co-occurrence of types 1 and 2 PrP\n                    res\n                   in sporadic Creutzfeldt-Jakob disease MM1. Am J Pathol 2011, 178: 1309\u20131315.","journal-title":"Am J Pathol"},{"key":"67_CR29","doi-asserted-by":"publisher","first-page":"30022","DOI":"10.1074\/jbc.M704597200","volume":"282","author":"A Kobayashi","year":"2007","unstructured":"Kobayashi A, Asano M, Mohri S, Kitamoto T: Cross-sequence transmission of sporadic Creutzfeldt-Jakob disease creates a new prion strain. J Biol Chem 2007, 282: 30022\u201330028.","journal-title":"J Biol Chem"},{"key":"67_CR30","volume-title":"The mouse brain in stereotaxic coordinates","author":"G Paxinos","year":"2012","unstructured":"Paxinos G, Franklin KBJ: The mouse brain in stereotaxic coordinates. 4th edition. London: Academic Press; 2012.","edition":"4"},{"key":"67_CR31","doi-asserted-by":"publisher","first-page":"619","DOI":"10.1111\/j.1440-1789.2008.00973.x","volume":"29","author":"A Kobayashi","year":"2009","unstructured":"Kobayashi A, Asano M, Mohri S, Kitamoto T: A traceback phenomenon can reveal the origin of prion infection. Neuropathology 2009, 29: 619\u2013624.","journal-title":"Neuropathology"},{"key":"67_CR32","doi-asserted-by":"publisher","first-page":"245","DOI":"10.1111\/j.1750-3639.2003.tb00025.x","volume":"13","author":"HA Kretzschmar","year":"2003","unstructured":"Kretzschmar HA, Sethi S, F\u00f6ldv\u00e1ri Z, Windl O, Querner V, Zerr I, et al.: Iatrogenic Creutzfeldt-Jakob disease with florid plaques. Brain Pathol 2003, 13: 245\u2013249.","journal-title":"Brain Pathol"},{"key":"67_CR33","doi-asserted-by":"publisher","first-page":"801","DOI":"10.1038\/353801b0","volume":"353","author":"K Doh-ura","year":"1991","unstructured":"Doh-ura K, Kitamoto T, Sakaki Y, Tateishi J: CJD discrepancy. Nature 1991, 353: 801\u2013802.","journal-title":"Nature"},{"key":"67_CR34","doi-asserted-by":"publisher","first-page":"3603","DOI":"10.1074\/jbc.M809254200","volume":"284","author":"M Hizume","year":"2009","unstructured":"Hizume M, Kobayashi A, Teruya K, Ohashi H, Ironside JW, Mohri S, et al.: Human prion protein (PrP) 219K is converted to PrP\n                    Sc\n                   but shows heterozygous inhibition in variant Creutzfeldt-Jakob disease infection\n. J Biol Chem 2009, 284: 3603\u20133609.","journal-title":"J Biol Chem"},{"key":"67_CR35","doi-asserted-by":"publisher","first-page":"27","DOI":"10.4161\/pri.3.1.8514","volume":"3","author":"A Kobayashi","year":"2009","unstructured":"Kobayashi A, Hizume M, Teruya K, Mohri S, Kitamoto T: Heterozygous inhibition in prion infection: the stone fence model\n. Prion 2009, 3: 27\u201330.","journal-title":"Prion"},{"key":"67_CR36","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.: 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\n. Brain 2010, 133: 3030\u20133042.","journal-title":"Brain"},{"key":"67_CR37","doi-asserted-by":"publisher","first-page":"609","DOI":"10.1111\/j.1440-1789.2008.00987.x","volume":"29","author":"M Yamada","year":"2009","unstructured":"Yamada M, Noguchi-Shinohara M, Hamaguchi T, Nozaki I, Kitamoto T, Sato T, et al.: Dura mater graft-associated Creutzfeldt-Jakob disease in Japan: clinicopathological and molecular characterization of the two distinct subtypes. Neuropathology 2009, 29: 609\u2013618.","journal-title":"Neuropathology"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/2051-5960-1-74.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,2]],"date-time":"2021-09-02T02:07:44Z","timestamp":1630548464000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/2051-5960-1-74"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,11,13]]},"references-count":37,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013,12]]}},"alternative-id":["67"],"URL":"https:\/\/doi.org\/10.1186\/2051-5960-1-74","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,11,13]]},"assertion":[{"value":"8 November 2013","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 November 2013","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"13 November 2013","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"74"}}