{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,14]],"date-time":"2026-10-14T17:40:45Z","timestamp":1791999645765,"version":"4.4.0"},"reference-count":42,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T00:00:00Z","timestamp":1754956800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"},{"start":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T00:00:00Z","timestamp":1754956800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0"}],"funder":[{"DOI":"10.13039\/100021080","name":"Creutzfeldt-Jakob Disease Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100021080","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Ottawa Jewish Community Foundation","award":["Alana Bodnoff Perelmutter Fund for Prion Disease Research"],"award-info":[{"award-number":["Alana Bodnoff Perelmutter Fund for Prion Disease Research"]}]},{"name":"Ottawa Jewish Community Foundation","award":["Alana Bodnoff Perelmutter Fund for Prion Disease Research"],"award-info":[{"award-number":["Alana Bodnoff Perelmutter Fund for Prion Disease Research"]}]},{"name":"CJD patient family donors"},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["K99\/R00 AG068359"],"award-info":[{"award-number":["K99\/R00 AG068359"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["5R01NS128226"],"award-info":[{"award-number":["5R01NS128226"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000030","name":"Centers for Disease Control and Prevention","doi-asserted-by":"publisher","award":["1NU38CK000486"],"award-info":[{"award-number":["1NU38CK000486"]}],"id":[{"id":"10.13039\/100000030","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["acta neuropathol commun"],"DOI":"10.1186\/s40478-025-02079-9","type":"journal-article","created":{"date-parts":[[2025,8,12]],"date-time":"2025-08-12T11:47:19Z","timestamp":1754999239000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Variably protease-sensitive prionopathy: mass spectrometry analysis of the pathogenic prion protein provides a new perspective"],"prefix":"10.1186","volume":"13","author":[{"given":"S.K.","family":"Nemani","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"X.","family":"Xiao","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Notari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"I.","family":"Cali","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"K.","family":"Lundberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"L.","family":"Cracco","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"B.S.","family":"Appleby","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"W.K.","family":"Surewicz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"V.L.","family":"Sim","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"P.","family":"Gambetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2025,8,12]]},"reference":[{"key":"2079_CR1","doi-asserted-by":"publisher","first-page":"1476","DOI":"10.1093\/emboj\/19.7.1476","volume":"19","author":"IC Almeida","year":"2000","unstructured":"Almeida IC, Camargo MM, Proc\u00f3pio DO, Silva LS, Mehlert A, Travassos LR, Gazzinelli RT, Ferguson MA (2000) Highly purified glycosylphosphatidylinositols from trypanosoma Cruzi are potent Proinflammatory agents. Embo J 19:1476\u20131485. https:\/\/doi.org\/10.1093\/emboj\/19.7.1476","journal-title":"Embo J"},{"key":"2079_CR2","doi-asserted-by":"publisher","first-page":"939","DOI":"10.1007\/s00216-012-6203-4","volume":"404","author":"M Bantscheff","year":"2012","unstructured":"Bantscheff M, Lemeer S, Savitski MM, Kuster B (2012) Quantitative mass spectrometry in proteomics: critical review update from 2007 to the present. Anal Bioanal Chem 404:939\u2013965. https:\/\/doi.org\/10.1007\/s00216-012-6203-4","journal-title":"Anal Bioanal Chem"},{"key":"2079_CR3","doi-asserted-by":"publisher","first-page":"579","DOI":"10.1016\/0003-9861(87)90380-8","volume":"258","author":"DC Bolton","year":"1987","unstructured":"Bolton DC, Bendheim PE, Marmorstein AD, Potempska A (1987) Isolation and structural studies of the intact scrapie agent protein. Arch Biochem Biophys 258:579\u2013590. https:\/\/doi.org\/10.1016\/0003-9861(87)90380-8","journal-title":"Arch Biochem Biophys"},{"key":"2079_CR4","doi-asserted-by":"publisher","first-page":"38280","DOI":"10.1038\/srep38280","volume":"7","author":"L Cracco","year":"2017","unstructured":"Cracco L, Notari S, Cali I, Sy MS, Chen SG, Cohen ML, Ghetti B, Appleby BS, Zou WQ, Caughey B et al (2017) Novel strain properties distinguishing sporadic prion diseases sharing prion protein genotype and prion type. Sci Rep 7:38280. https:\/\/doi.org\/10.1038\/srep38280","journal-title":"Sci Rep"},{"key":"2079_CR5","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1186\/s40478-019-0734-2","volume":"7","author":"L Cracco","year":"2019","unstructured":"Cracco L, Xiao X, Nemani SK, Lavrich J, Cali I, Ghetti B, Notari S, Surewicz WK, Gambetti P (2019) Gerstmann-Str\u00e4ussler-Scheinker disease revisited: accumulation of covalently-linked multimers of internal prion protein fragments. Acta Neuropathol Commun 7:85. https:\/\/doi.org\/10.1186\/s40478-019-0734-2","journal-title":"Acta Neuropathol Commun"},{"key":"2079_CR6","doi-asserted-by":"publisher","first-page":"11247","DOI":"10.1074\/jbc.M407006200","volume":"280","author":"C F\u00e9raudet","year":"2005","unstructured":"F\u00e9raudet C, Morel N, Simon S, Volland H, Frobert Y, Cr\u00e9minon C, Vilette D, Lehmann S, Grassi J (2005) Screening of 145 anti-PrP monoclonal antibodies for their capacity to inhibit PrPSc replication in infected cells. J Biol Chem 280:11247\u201311258. https:\/\/doi.org\/10.1074\/jbc.M407006200","journal-title":"J Biol Chem"},{"key":"2079_CR7","doi-asserted-by":"publisher","first-page":"389","DOI":"10.1016\/bs.pmbts.2017.06.012","volume":"150","author":"M Fiorini","year":"2017","unstructured":"Fiorini M, Bongianni M, Monaco S, Zanusso G (2017) Biochemical characterization of prions. Prog Mol Biol Transl Sci 150:389\u2013407. https:\/\/doi.org\/10.1016\/bs.pmbts.2017.06.012","journal-title":"Prog Mol Biol Transl Sci"},{"key":"2079_CR8","doi-asserted-by":"publisher","first-page":"353","DOI":"10.1007\/978-3-031-20565-1_18","volume-title":"Prions and diseases","author":"P Gambetti","year":"2023","unstructured":"Gambetti P, Cali I (2023) Human sporadic prion diseases. In: Zou W-Q, Gambetti P (eds) Prions and diseases. Springer International Publishing, City, pp 353\u2013373"},{"key":"2079_CR9","doi-asserted-by":"publisher","first-page":"697","DOI":"10.1002\/ana.21420","volume":"63","author":"P Gambetti","year":"2008","unstructured":"Gambetti P, Dong Z, Yuan J, Xiao X, Zheng M, Alshekhlee A, Castellani R, Cohen M, Barria MA, Gonzalez-Romero D et al (2008) A novel human disease with abnormal prion protein sensitive to protease. Ann Neurol 63:697\u2013708. https:\/\/doi.org\/10.1002\/ana.21420","journal-title":"Ann Neurol"},{"key":"2079_CR10","doi-asserted-by":"publisher","first-page":"213","DOI":"10.1093\/bmb\/66.1.213","volume":"66","author":"P Gambetti","year":"2003","unstructured":"Gambetti P, Kong Q, Zou W, Parchi P, Chen SG (2003) Sporadic and Familial CJD: classification and characterisation. Br Med Bull 66:213\u2013239. https:\/\/doi.org\/10.1093\/bmb\/66.1.213","journal-title":"Br Med Bull"},{"key":"2079_CR11","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1007\/s00401-022-02461-0","volume":"144","author":"GI Hallinan","year":"2022","unstructured":"Hallinan GI, Ozcan KA, Hoq MR, Cracco L, Vago FS, Bharath SR, Li D, Jacobsen M, Doud EH, Mosley AL et al (2022) Cryo-EM structures of prion protein filaments from Gerstmann-Str\u00e4ussler-Scheinker disease. Acta Neuropathol 144:509\u2013520. https:\/\/doi.org\/10.1007\/s00401-022-02461-0","journal-title":"Acta Neuropathol"},{"key":"2079_CR12","doi-asserted-by":"publisher","first-page":"1102","DOI":"10.1093\/brain\/aws366","volume":"136","author":"MW Head","year":"2013","unstructured":"Head MW, Yull HM, Ritchie DL, Langeveld JP, Fletcher NA, Knight RS, Ironside JW (2013) Variably protease-sensitive prionopathy in the UK: a retrospective review 1991\u20132008. Brain 136:1102\u20131115. https:\/\/doi.org\/10.1093\/brain\/aws366","journal-title":"Brain"},{"key":"2079_CR13","doi-asserted-by":"publisher","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, Wisniewski HM, Diringer H (1987) Mouse polyclonal and monoclonal antibody to scrapie-associated fibril proteins. J Virol 61:3688\u20133693. https:\/\/doi.org\/10.1128\/jvi.61.12.3688-3693.1987","journal-title":"J Virol"},{"key":"2079_CR14","doi-asserted-by":"publisher","first-page":"7789","DOI":"10.1074\/jbc.RA120.013444","volume":"295","author":"A Kobayashi","year":"2020","unstructured":"Kobayashi A, Hirata T, Nishikaze T, Ninomiya A, Maki Y, Takada Y, Kitamoto T, Kinoshita T (2020) \u03b12,3 linkage of Sialic acid to a GPI anchor and an unpredicted GPI attachment site in human prion protein. J Biol Chem 295:7789\u20137798. https:\/\/doi.org\/10.1074\/jbc.RA120.013444","journal-title":"J Biol Chem"},{"key":"2079_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. https:\/\/doi.org\/10.1128\/jvi.02387-09","journal-title":"J Virol"},{"key":"2079_CR16","doi-asserted-by":"publisher","first-page":"19","DOI":"10.1186\/1746-6148-2-19","volume":"2","author":"JP Langeveld","year":"2006","unstructured":"Langeveld JP, Jacobs JG, Erkens JH, Bossers A, van Zijderveld FG, van Keulen LJ (2006) Rapid and discriminatory diagnosis of scrapie and BSE in retro-pharyngeal lymph nodes of sheep. BMC Vet Res 2:19. https:\/\/doi.org\/10.1186\/1746-6148-2-19","journal-title":"BMC Vet Res"},{"key":"2079_CR17","doi-asserted-by":"publisher","first-page":"567","DOI":"10.1006\/jmbi.2000.3986","volume":"301","author":"R Li","year":"2000","unstructured":"Li R, Liu T, Wong BS, Pan T, Morillas M, Swietnicki W, O\u2019Rourke K, Gambetti P, Surewicz WK, Sy MS (2000) Identification of an epitope in the C terminus of normal prion protein whose expression is modulated by binding events in the N terminus. J Mol Biol 301:567\u2013573. https:\/\/doi.org\/10.1006\/jmbi.2000.3986","journal-title":"J Mol Biol"},{"key":"2079_CR18","doi-asserted-by":"publisher","DOI":"10.1101\/2024.12.12.24318867","author":"Y Lian","year":"2024","unstructured":"Lian Y, Kotobelli K, Hall S, Talkowski ME, O\u2019Donnell-Luria A, Vallabh SM, Appleby BS, Minikel EV (2024) Search for a genetic cause of variably protease-sensitive prionopathy. MedRxiv: Doi. https:\/\/doi.org\/10.1101\/2024.12.12.24318867","journal-title":"MedRxiv: Doi"},{"key":"2079_CR19","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1074\/mcp.M114.042242","volume":"14","author":"KC Lundberg","year":"2015","unstructured":"Lundberg KC, Fritz Y, Johnston A, Foster AM, Baliwag J, Gudjonsson JE, Schlatzer D, Gokulrangan G, McCormick TS, Chance MR et al (2015) Proteomics of skin proteins in psoriasis: from discovery and verification in a mouse model to confirmation in humans. Mol Cell Proteom 14:109\u2013119. https:\/\/doi.org\/10.1074\/mcp.M114.042242","journal-title":"Mol Cell Proteom"},{"key":"2079_CR20","doi-asserted-by":"publisher","first-page":"2839","DOI":"10.1073\/pnas.91.7.2839","volume":"91","author":"L Monari","year":"1994","unstructured":"Monari L, Chen SG, Brown P, Parchi P, Petersen RB, Mikol J, Gray F, Cortelli P, Montagna P, Ghetti B et al (1994) Fatal Familial insomnia and Familial Creutzfeldt-Jakob disease: different prion proteins determined by a DNA polymorphism. Proc Natl Acad Sci U S A 91:2839\u20132842. https:\/\/doi.org\/10.1073\/pnas.91.7.2839","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2079_CR21","doi-asserted-by":"publisher","first-page":"261","DOI":"10.1038\/msb.2009.13","volume":"5","author":"ES Nakayasu","year":"2009","unstructured":"Nakayasu ES, Yashunsky DV, Nohara LL, Torrecilhas AC, Nikolaev AV, Almeida IC (2009) GPIomics: global analysis of glycosylphosphatidylinositol-anchored molecules of trypanosoma Cruzi. Mol Syst Biol 5:261. https:\/\/doi.org\/10.1038\/msb.2009.13","journal-title":"Mol Syst Biol"},{"key":"2079_CR22","doi-asserted-by":"publisher","first-page":"85","DOI":"10.1186\/s40478-020-00966-x","volume":"8","author":"SK Nemani","year":"2020","unstructured":"Nemani SK, Xiao X, Cali I, Cracco L, Puoti G, Nigro M, Lavrich J, Bharara Singh A, Appleby BS, Sim VL et al (2020) A novel mechanism of phenotypic heterogeneity in Creutzfeldt-Jakob disease. Acta Neuropathol Commun 8:85. https:\/\/doi.org\/10.1186\/s40478-020-00966-x","journal-title":"Acta Neuropathol Commun"},{"key":"2079_CR23","doi-asserted-by":"publisher","first-page":"175","DOI":"10.1016\/B978-0-444-63945-5.00010-6","volume":"153","author":"S Notari","year":"2018","unstructured":"Notari S, Appleby BS, Gambetti P (2018) Variably protease-sensitive prionopathy. Handb Clin Neurol 153:175\u2013190. https:\/\/doi.org\/10.1016\/b978-0-444-63945-5.00010","journal-title":"Handb Clin Neurol"},{"key":"2079_CR24","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, Gambetti P, Kretzschmar HA, Parchi P (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. https:\/\/doi.org\/10.1074\/jbc.M313220200","journal-title":"J Biol Chem"},{"key":"2079_CR25","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, Ghetti B, Langeveld JP, Zou WQ, Gambetti P et al (2008) Characterization of truncated forms of abnormal prion protein in Creutzfeldt-Jakob disease. J Biol Chem 283:30557\u201330565. https:\/\/doi.org\/10.1074\/jbc.M801877200","journal-title":"J Biol Chem"},{"key":"2079_CR26","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, Kopp N, Schulz-Schaeffer WJ, Kretzschmar HA, Head MW et al (2000) Genetic influence on the structural variations of the abnormal prion protein. Proc Natl Acad Sci U S A 97:10168\u201310172. https:\/\/doi.org\/10.1073\/pnas.97.18.10168","journal-title":"Proc Natl Acad Sci U S A"},{"key":"2079_CR27","doi-asserted-by":"publisher","first-page":"2201","DOI":"10.1016\/s0002-9440(10)64692-5","volume":"158","author":"P Piccardo","year":"2001","unstructured":"Piccardo P, Liepnieks JJ, William A, Dlouhy SR, Farlow MR, Young K, Nochlin D, Bird TD, Nixon RR, Ball MJ et al (2001) Prion proteins with different conformations accumulate in Gerstmann-Str\u00e4ussler-Scheinker disease caused by A117V and F198S mutations. Am J Pathol 158:2201\u20132207. https:\/\/doi.org\/10.1016\/s0002-9440(10)64692-5","journal-title":"Am J Pathol"},{"key":"2079_CR28","doi-asserted-by":"publisher","first-page":"e66405","DOI":"10.1371\/journal.pone.0066405","volume":"8","author":"L Pirisinu","year":"2013","unstructured":"Pirisinu L, Nonno R, Esposito E, Benestad SL, Gambetti P, Agrimi U, Zou WQ (2013) Small ruminant nor98 prions share biochemical features with human gerstmann-str\u00e4ussler-scheinker disease and variably protease-sensitive prionopathy. PLoS ONE 8:e66405. https:\/\/doi.org\/10.1371\/journal.pone.0066405","journal-title":"PLoS ONE"},{"key":"2079_CR29","doi-asserted-by":"publisher","first-page":"621","DOI":"10.1089\/ars.2022.0199","volume":"39","author":"D Seth","year":"2023","unstructured":"Seth D, Stomberski CT, McLaughlin PJ, Premont RT, Lundberg K, Stamler JS (2023) Comparison of the nitric oxide synthase interactomes and S-Nitroso-Proteomes: furthering the case for enzymatic S-Nitrosylation. Antioxid Redox Signal 39:621\u2013634. https:\/\/doi.org\/10.1089\/ars.2022.0199","journal-title":"Antioxid Redox Signal"},{"key":"2079_CR30","doi-asserted-by":"publisher","unstructured":"Song F, Kovac V, Mohammadi B, Littau JL, Scharfenberg F, Matamoros Angles A, Vanni I, Shafiq M, Orge L, Galliciotti G et al (2024) Cleavage site-directed antibodies reveal the prion protein in humans is shed by ADAM10 at Y226 and associates with misfolded protein deposits in neurodegenerative diseases. Acta Neuropathol 148(2). https:\/\/doi.org\/10.1007\/s00401-024-02763-5","DOI":"10.1007\/s00401-024-02763-5"},{"key":"2079_CR31","doi-asserted-by":"publisher","first-page":"2301","DOI":"10.1038\/nprot.2016.136","volume":"11","author":"S Tyanova","year":"2016","unstructured":"Tyanova S, Temu T, Cox J (2016) The MaxQuant computational platform for mass spectrometry-based shotgun proteomics. Nat Protoc 11:2301\u20132319. https:\/\/doi.org\/10.1038\/nprot.2016.136","journal-title":"Nat Protoc"},{"key":"2079_CR32","doi-asserted-by":"publisher","first-page":"486","DOI":"10.1111\/neup.12911","volume":"43","author":"A Uchino","year":"2023","unstructured":"Uchino A, Saito Y, Oonuma S, Murayama S, Yagishita S, Kitamoto T, Hasegawa K (2023) An autopsy case of variably protease-sensitive prionopathy with met\/met homogeneity at codon 129. Neuropathology 43:486\u2013495. https:\/\/doi.org\/10.1111\/neup.12911","journal-title":"Neuropathology"},{"key":"2079_CR33","doi-asserted-by":"publisher","first-page":"425","DOI":"10.1007\/978-3-031-20565-1_20","volume-title":"Prions and diseases","author":"Z Wang","year":"2023","unstructured":"Wang Z, Yuan J, Gilliland T, Gerasimenko M, Shah SZA, Zou W-Q (2023) Glycoform-Selective prions in sporadic and genetic variably Protease-Sensitive prionopathies. In: Zou W-Q, Gambetti P (eds) Prions and diseases. Springer International Publishing, City, pp 425\u2013445"},{"key":"2079_CR34","doi-asserted-by":"publisher","first-page":"e58786","DOI":"10.1371\/journal.pone.0058786","volume":"8","author":"X Xiao","year":"2013","unstructured":"Xiao X, Yuan J, Ha\u00efk S, Cali I, Zhan Y, Moudjou M, Li B, Laplanche JL, Laude H, Langeveld J et al (2013) Glycoform-selective prion formation in sporadic and Familial forms of prion disease. PLoS ONE 8:e58786. https:\/\/doi.org\/10.1371\/journal.pone.0058786","journal-title":"PLoS ONE"},{"key":"2079_CR35","doi-asserted-by":"publisher","first-page":"34848","DOI":"10.1074\/jbc.M602238200","volume":"281","author":"J Yuan","year":"2006","unstructured":"Yuan J, Xiao X, McGeehan J, Dong Z, Cali I, Fujioka H, Kong Q, Kneale G, Gambetti P, Zou WQ (2006) Insoluble aggregates and protease-resistant conformers of prion protein in uninfected human brains. J Biol Chem 281:34848\u201334858. https:\/\/doi.org\/10.1074\/jbc.M602238200","journal-title":"J Biol Chem"},{"key":"2079_CR36","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, Head MW (2006) Detection of type 1 prion protein in variant Creutzfeldt-Jakob disease. Am J Pathol 168:151\u2013157. https:\/\/doi.org\/10.2353\/ajpath.2006.050766","journal-title":"Am J Pathol"},{"key":"2079_CR37","doi-asserted-by":"publisher","first-page":"595","DOI":"10.1001\/archneur.64.4.595","volume":"64","author":"G Zanusso","year":"2007","unstructured":"Zanusso G, Polo A, Farinazzo A, Nonno R, Cardone F, Di Bari M, Ferrari S, Principe S, Gelati M, Fasoli E et al (2007) Novel prion protein conformation and glycotype in Creutzfeldt-Jakob disease. Arch Neurol 64:595\u2013599. https:\/\/doi.org\/10.1001\/archneur.64.4.595","journal-title":"Arch Neurol"},{"key":"2079_CR38","doi-asserted-by":"publisher","first-page":"155","DOI":"10.1016\/b978-0-444-63945-5.00009-x","volume":"153","author":"I Zerr","year":"2018","unstructured":"Zerr I, Parchi P (2018) Sporadic Creutzfeldt-Jakob disease. Handb Clin Neurol 153:155\u2013174. https:\/\/doi.org\/10.1016\/b978-0-444-63945-5.00009-x","journal-title":"Handb Clin Neurol"},{"key":"2079_CR39","doi-asserted-by":"publisher","unstructured":"Zhang W, Xiao X, Ding M, Yuan J, Foutz A, Moudjou M, Kitamoto T, Langeveld JPM, Cui L, Zou WQ (2021) Further characterization of Glycoform-Selective prions of variably Protease-Sensitive prionopathy. Pathogens 10. https:\/\/doi.org\/10.3390\/pathogens10050513","DOI":"10.3390\/pathogens10050513"},{"key":"2079_CR40","doi-asserted-by":"publisher","first-page":"40429","DOI":"10.1074\/jbc.M308550200","volume":"278","author":"WQ Zou","year":"2003","unstructured":"Zou WQ, Capellari S, Parchi P, Sy MS, 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. https:\/\/doi.org\/10.1074\/jbc.M308550200","journal-title":"J Biol Chem"},{"key":"2079_CR41","doi-asserted-by":"publisher","first-page":"457","DOI":"10.3390\/pathogens2030457","volume":"2","author":"WQ Zou","year":"2013","unstructured":"Zou WQ, Gambetti P, Xiao X, Yuan J, Langeveld J, Pirisinu L (2013) Prions in variably protease-sensitive prionopathy: an update. Pathogens 2:457\u2013471. https:\/\/doi.org\/10.3390\/pathogens2030457","journal-title":"Pathogens"},{"key":"2079_CR42","doi-asserted-by":"publisher","first-page":"162","DOI":"10.1002\/ana.22094","volume":"68","author":"WQ Zou","year":"2010","unstructured":"Zou WQ, Puoti G, Xiao X, Yuan J, Qing L, Cali I, Shimoji M, Langeveld JP, Castellani R, Notari S et al (2010) Variably protease-sensitive prionopathy: a new sporadic disease of the prion protein. Ann Neurol 68:162\u2013172. https:\/\/doi.org\/10.1002\/ana.22094","journal-title":"Ann Neurol"}],"container-title":["Acta Neuropathologica Communications"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-025-02079-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1186\/s40478-025-02079-9\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/s40478-025-02079-9.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,9,9]],"date-time":"2025-09-09T11:58:04Z","timestamp":1757419084000},"score":1,"resource":{"primary":{"URL":"https:\/\/actaneurocomms.biomedcentral.com\/articles\/10.1186\/s40478-025-02079-9"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,8,12]]},"references-count":42,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2025,12]]}},"alternative-id":["2079"],"URL":"https:\/\/doi.org\/10.1186\/s40478-025-02079-9","relation":{},"ISSN":["2051-5960"],"issn-type":[{"value":"2051-5960","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,8,12]]},"assertion":[{"value":"9 May 2025","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"17 July 2025","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"12 August 2025","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"27 August 2025","order":4,"name":"change_date","label":"Change Date","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"Update","order":5,"name":"change_type","label":"Change Type","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The original online version of this article was revised: Legends of supplementary figures and tables were missing from this supplementary material and it has been updated.","order":6,"name":"change_details","label":"Change Details","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"The human brain tissues used in the current study were provided by NPDPSC. All the procedures were performed with institutional review board of University Hospitals Cleveland Medical Center (#5\u201314\u2009\u2212\u200909) approval and as per regulations of 1964 Declaration of Helsinki.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}},{"value":"Not applicable.","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Consent for publication"}},{"value":"The authors declare no competing interests.","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing interests"}}],"article-number":"172"}}