{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,9,6]],"date-time":"2024-09-06T10:52:56Z","timestamp":1725619976032},"reference-count":99,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2010,6,10]],"date-time":"2010-06-10T00:00:00Z","timestamp":1276128000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Acta Neuropathol"],"published-print":{"date-parts":[[2011,1]]},"DOI":"10.1007\/s00401-010-0708-8","type":"journal-article","created":{"date-parts":[[2010,6,9]],"date-time":"2010-06-09T05:33:32Z","timestamp":1276061612000},"page":"135-143","source":"Crossref","is-referenced-by-count":16,"title":["The application of in vitro cell-free conversion systems to human prion diseases"],"prefix":"10.1007","volume":"121","author":[{"given":"Michael","family":"Jones","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alexander H.","family":"Peden","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark W.","family":"Head","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"James W.","family":"Ironside","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2010,6,10]]},"reference":[{"key":"708_CR1","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1042\/bse0330133","volume":"33","author":"A Aguzzi","year":"1998","unstructured":"Aguzzi A, Klein MA, Musahl C et al (1998) Use of brain grafts to study the pathogenesis of prion diseases. Essays Biochem 33:133\u2013147","journal-title":"Essays Biochem"},{"key":"708_CR2","doi-asserted-by":"crossref","unstructured":"Aguzzi A, Sigurdson C, Heikenwalder M (2007) Molecular mechanisms of prion pathogenesis. Annu Rev Pathol","DOI":"10.1146\/annurev.pathol.3.121806.154326"},{"key":"708_CR3","doi-asserted-by":"crossref","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 et al (2006) vCJD prion acquires altered virulence through trans-species infection. Biochem Biophys Res Commun 342:293\u2013299","journal-title":"Biochem Biophys Res Commun"},{"key":"708_CR4","doi-asserted-by":"crossref","first-page":"645","DOI":"10.1038\/nmeth1066","volume":"4","author":"R Atarashi","year":"2007","unstructured":"Atarashi R, Moore RA, Sim VL et al (2007) Ultrasensitive detection of scrapie prion protein using seeded conversion of recombinant prion protein. Nat Methods 4:645\u2013650","journal-title":"Nat Methods"},{"key":"708_CR5","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/nmeth0308-211","volume":"5","author":"R Atarashi","year":"2008","unstructured":"Atarashi R, Wilham JM, Christensen L et al (2008) Simplified ultrasensitive prion detection by recombinant PrP conversion with shaking. Nat Methods 5:211\u2013212","journal-title":"Nat Methods"},{"key":"708_CR6","doi-asserted-by":"crossref","first-page":"e1000421","DOI":"10.1371\/journal.ppat.1000421","volume":"5","author":"MA Barria","year":"2009","unstructured":"Barria MA, Mukherjee A, Gonzalez-Romero D, Morales R, Soto C (2009) De novo generation of infectious prions in vitro produces a new disease phenotype. PLoS Pathog 5:e1000421","journal-title":"PLoS Pathog"},{"key":"708_CR7","doi-asserted-by":"crossref","first-page":"698","DOI":"10.1038\/375698a0","volume":"375","author":"RA Bessen","year":"1995","unstructured":"Bessen RA, Kocisko DA, Raymond GJ et al (1995) Non-genetic propagation of strain-specific properties of scrapie prion protein. Nature 375:698\u2013700","journal-title":"Nature"},{"key":"708_CR8","doi-asserted-by":"crossref","first-page":"12207","DOI":"10.1073\/pnas.0404650101","volume":"101","author":"J Bieschke","year":"2004","unstructured":"Bieschke J, Weber P, Sarafoff N et al (2004) Autocatalytic self-propagation of misfolded prion protein. Proc Natl Acad Sci USA 101:12207\u201312211","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR9","doi-asserted-by":"crossref","first-page":"393","DOI":"10.1016\/S1474-4422(06)70413-6","volume":"5","author":"MT Bishop","year":"2006","unstructured":"Bishop MT, Hart P, Aitchison L et al (2006) Predicting susceptibility and incubation time of human-to-human transmission of vCJD. Lancet Neurol 5:393\u2013398","journal-title":"Lancet Neurol"},{"key":"708_CR10","doi-asserted-by":"crossref","first-page":"4931","DOI":"10.1073\/pnas.94.10.4931","volume":"94","author":"A Bossers","year":"1997","unstructured":"Bossers A, Belt PBGM, Raymond GJ et al (1997) Scrapie susceptibility-linked polymorphisms modulate the in vitro conversion of sheep prion protein to protease-resistant forms. Proc Natl Acad Sci USA 94:4931\u20134936","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR11","doi-asserted-by":"crossref","first-page":"1407","DOI":"10.1128\/JVI.74.3.1407-1414.2000","volume":"74","author":"A Bossers","year":"2000","unstructured":"Bossers A, de Vries R, Smits MA (2000) Susceptibility of sheep for scrapie as assessed by in vitro conversion of nine naturally occurring variants of PrP. J Virol 74:1407\u20131414","journal-title":"J Virol"},{"key":"708_CR12","doi-asserted-by":"crossref","first-page":"339","DOI":"10.1038\/379339a0","volume":"379","author":"S Brandner","year":"1996","unstructured":"Brandner S, Isenmann S, Raeber A et al (1996) Normal host prion protein necessary for scrapie-induced neurotoxicity. Nature 379:339\u2013343","journal-title":"Nature"},{"key":"708_CR13","doi-asserted-by":"crossref","first-page":"13148","DOI":"10.1073\/pnas.93.23.13148","volume":"93","author":"S Brandner","year":"1996","unstructured":"Brandner S, Raeber A, Sailer A et al (1996) Normal host prion protein (PrPC) is required for scrapie spread within the central nervous system. Proc Natl Acad Sci USA 93:13148\u201313151","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR14","doi-asserted-by":"crossref","first-page":"498","DOI":"10.1038\/39057","volume":"389","author":"ME Bruce","year":"1997","unstructured":"Bruce ME, Will RG, Ironside JW et al (1997) Transmissions to mice indicate that \u2018new variant\u2019 CJD is caused by the BSE agent. Nature 389:498\u2013501","journal-title":"Nature"},{"key":"708_CR15","first-page":"287","volume-title":"Neurodegeneration: the molecular pathology of dementia and movement disorders","author":"H Budka","year":"2003","unstructured":"Budka H, Head MW, Ironside JW et al (2003) Sporadic Creutzfeldt\u2013Jakob disease. In: Dickson DW et al (eds) Neurodegeneration: the molecular pathology of dementia and movement disorders. ISN Neuropath Press, Basel, pp 287\u2013297"},{"key":"708_CR16","doi-asserted-by":"crossref","first-page":"103","DOI":"10.1016\/j.vetmic.2006.06.016","volume":"117","author":"A Buschmann","year":"2006","unstructured":"Buschmann A, Gretzschel A, Biacabe AG et al (2006) Atypical BSE in Germany\u2014proof of transmissibility and biochemical characterization. Vet Microbiol 117:103\u2013116","journal-title":"Vet Microbiol"},{"key":"708_CR17","doi-asserted-by":"crossref","first-page":"2643","DOI":"10.1093\/brain\/awp196","volume":"132","author":"I Cali","year":"2009","unstructured":"Cali I, Castellani R, Alshekhlee A et al (2009) Co-existence of scrapie prion protein types 1 and 2 in sporadic Creutzfeldt\u2013Jakob disease: its effect on the phenotype and prion-type characteristics. Brain 132:2643\u20132658","journal-title":"Brain"},{"key":"708_CR18","doi-asserted-by":"crossref","first-page":"2557","DOI":"10.1038\/emboj.2008.181","volume":"27","author":"J Castilla","year":"2008","unstructured":"Castilla J, Morales R, Saa P et al (2008) Cell-free propagation of prion strains. EMBO J 27:2557\u20132566","journal-title":"EMBO J"},{"key":"708_CR19","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.cell.2005.02.011","volume":"121","author":"J Castilla","year":"2005","unstructured":"Castilla J, Saa P, Hetz C, Soto C (2005) In vitro generation of infectious scrapie prions. Cell 121:195\u2013206","journal-title":"Cell"},{"key":"708_CR20","doi-asserted-by":"crossref","first-page":"3","DOI":"10.1016\/S0076-6879(06)12001-7","volume":"412","author":"J Castilla","year":"2006","unstructured":"Castilla J, Saa P, Morales R et al (2006) Protein misfolding cyclic amplification for diagnosis and prion propagation studies. Methods Enzymol 412:3\u201321","journal-title":"Methods Enzymol"},{"key":"708_CR21","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1038\/nm1286","volume":"11","author":"J Castilla","year":"2005","unstructured":"Castilla J, Saa P, Soto C (2005) Detection of prions in blood. Nat Med 11:982\u2013985","journal-title":"Nat Med"},{"key":"708_CR22","doi-asserted-by":"crossref","first-page":"803","DOI":"10.1038\/nature05294","volume":"443","author":"B Caughey","year":"2006","unstructured":"Caughey B, Baron GS (2006) Prions and their partners in crime. Nature 443:803\u2013810","journal-title":"Nature"},{"key":"708_CR23","doi-asserted-by":"crossref","first-page":"122","DOI":"10.1016\/S0076-6879(99)09011-4","volume":"309","author":"B Caughey","year":"1999","unstructured":"Caughey B, Horiuchi M, Demaimay R, Raymond GJ (1999) Assays of protease-resistant prion protein and its formation. Methods Enzymol 309:122\u2013133","journal-title":"Methods Enzymol"},{"key":"708_CR24","doi-asserted-by":"crossref","first-page":"807","DOI":"10.1016\/1074-5521(95)90087-X","volume":"2","author":"B Caughey","year":"1995","unstructured":"Caughey B, Kocisko DA, Raymond GJ, Lansbury PT Jr (1995) Aggregates of scrapie-associated prion protein induce the cell-free conversion of protease-sensitive prion protein to the protease-resistant state. Chem Biol 2:807\u2013817","journal-title":"Chem Biol"},{"key":"708_CR25","doi-asserted-by":"crossref","first-page":"4107","DOI":"10.1128\/JVI.71.5.4107-4110.1997","volume":"71","author":"B Caughey","year":"1997","unstructured":"Caughey B, Raymond GJ, Kocisko DA, Lansbury PT Jr (1997) Scrapie infectivity correlates with converting activity, protease resistance, and aggregation of scrapie-associated prion protein in guanidine denaturation studies. J Virol 71:4107\u20134110","journal-title":"J Virol"},{"key":"708_CR26","doi-asserted-by":"crossref","first-page":"12117","DOI":"10.1073\/pnas.95.21.12117","volume":"95","author":"WS Caughey","year":"1998","unstructured":"Caughey WS, Raymond LD, Horiuchi M, Caughey B (1998) Inhibition of protease-resistant prion protein formation by porphyrins and phthalocyanines. Proc Natl Acad Sci USA 95:12117\u201312122","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR27","doi-asserted-by":"crossref","first-page":"13203","DOI":"10.1074\/jbc.273.21.13203","volume":"273","author":"J Chabry","year":"1998","unstructured":"Chabry J, Caughey B, Chesebro B (1998) Specific inhibition of in vitro formation of protease-resistant prion protein by synthetic peptides. J Biol Chem 273:13203\u201313207","journal-title":"J Biol Chem"},{"key":"708_CR28","doi-asserted-by":"crossref","first-page":"6245","DOI":"10.1128\/JVI.73.8.6245-6250.1999","volume":"73","author":"J Chabry","year":"1999","unstructured":"Chabry J, Priola SA, Wehrly K et al (1999) Species-independent inhibition of abnormal prion protein (PrP) formation by a peptide containing a conserved PrP sequence. J Virol 73:6245\u20136250","journal-title":"J Virol"},{"key":"708_CR29","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1016\/j.tibs.2009.03.001","volume":"34","author":"O Chakrabarti","year":"2009","unstructured":"Chakrabarti O, Ashok A, Hegde RS (2009) Prion protein biosynthesis and its emerging role in neurodegeneration. Trends Biochem Sci 34:287\u2013295","journal-title":"Trends Biochem Sci"},{"key":"708_CR30","doi-asserted-by":"crossref","first-page":"12633","DOI":"10.1073\/pnas.192137799","volume":"99","author":"PY Chen","year":"2002","unstructured":"Chen PY, Lin CC, Chang YT, Lin SC, Chan SI (2002) One O-linked sugar can affect the coil-to-beta structural transition of the prion peptide. Proc Natl Acad Sci USA 99:12633\u201312638","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR31","doi-asserted-by":"crossref","first-page":"20417","DOI":"10.1073\/pnas.0910350106","volume":"106","author":"DW Colby","year":"2009","unstructured":"Colby DW, Giles K, Legname G et al (2009) Design and construction of diverse mammalian prion strains. Proc Natl Acad Sci USA 106:20417\u201320422","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR32","doi-asserted-by":"crossref","first-page":"20914","DOI":"10.1073\/pnas.0710152105","volume":"104","author":"DW Colby","year":"2007","unstructured":"Colby DW, Zhang Q, Wang S et al (2007) Prion detection by an amyloid seeding assay. Proc Natl Acad Sci USA 104:20914\u201320919","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR33","doi-asserted-by":"crossref","first-page":"930","DOI":"10.1126\/science.1138718","volume":"318","author":"J Collinge","year":"2007","unstructured":"Collinge J, Clarke AR (2007) A general model of prion strains and their pathogenicity. Science 318:930\u2013936","journal-title":"Science"},{"key":"708_CR34","doi-asserted-by":"crossref","first-page":"13938","DOI":"10.1073\/pnas.94.25.13938","volume":"94","author":"SK DebBurman","year":"1997","unstructured":"DebBurman SK, Raymond GJ, Caughey B, Lindquist S (1997) Chaperone-supervised conversion of prion protein to its protease-resistant form. Proc Natl Acad Sci USA 94:13938\u201313943","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR35","doi-asserted-by":"crossref","first-page":"9741","DOI":"10.1073\/pnas.0702662104","volume":"104","author":"NR Deleault","year":"2007","unstructured":"Deleault NR, Harris BT, Rees JR, Supattapone S (2007) From the cover: formation of native prions from minimal components in vitro. Proc Natl Acad Sci USA 104:9741\u20139746","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR36","doi-asserted-by":"crossref","first-page":"2534","DOI":"10.1046\/j.1471-4159.1998.71062534.x","volume":"71","author":"R Demaimay","year":"1998","unstructured":"Demaimay R, Harper J, Gordon H et al (1998) Structural aspects of Congo red as an inhibitor of protease-resistant prion protein formation. J Neurochem 71:2534\u20132541","journal-title":"J Neurochem"},{"key":"708_CR37","doi-asserted-by":"crossref","first-page":"3753","DOI":"10.1099\/vir.0.81590-0","volume":"87","author":"M Eiden","year":"2006","unstructured":"Eiden M, Palm GJ, Hinrichs W et al (2006) Synergistic and strain-specific effects of bovine spongiform encephalopathy and scrapie prions in the cell-free conversion of recombinant prion protein. J Gen Virol 87:3753\u20133761","journal-title":"J Gen Virol"},{"key":"708_CR38","doi-asserted-by":"crossref","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","journal-title":"Br Med Bull"},{"key":"708_CR39","doi-asserted-by":"crossref","first-page":"e1000139","DOI":"10.1371\/journal.ppat.1000139","volume":"4","author":"KM Green","year":"2008","unstructured":"Green KM, Castilla J, Seward TS et al (2008) Accelerated high fidelity prion amplification within and across prion species barriers. PLoS Pathog 4:e1000139","journal-title":"PLoS Pathog"},{"key":"708_CR40","doi-asserted-by":"crossref","first-page":"851","DOI":"10.1002\/ana.20127","volume":"55","author":"MW Head","year":"2004","unstructured":"Head MW, Bunn TJ, Bishop MT et al (2004) Prion protein heterogeneity in sporadic but not variant Creutzfeldt\u2013Jakob disease: UK cases 1991\u20132002. Ann Neurol 55:851\u2013859","journal-title":"Ann Neurol"},{"key":"708_CR41","doi-asserted-by":"crossref","first-page":"2457","DOI":"10.1097\/00001756-199808030-00006","volume":"9","author":"LM Herrmann","year":"1998","unstructured":"Herrmann LM, Caughey B (1998) The importance of the disulfide bond in prion protein conversion. Neuroreport 9:2457\u20132461","journal-title":"Neuroreport"},{"issue":"Pt 1","key":"708_CR42","doi-asserted-by":"crossref","first-page":"11","DOI":"10.1099\/0022-1317-80-1-11","volume":"80","author":"AF Hill","year":"1999","unstructured":"Hill AF, Antoniou M, Collinge J (1999) Protease-resistant prion protein produced in vitro lacks detectable infectivity. J Gen Virol 80(Pt 1):11\u201314","journal-title":"J Gen Virol"},{"key":"708_CR43","doi-asserted-by":"crossref","first-page":"5836","DOI":"10.1073\/pnas.110523897","volume":"97","author":"M Horiuchi","year":"2000","unstructured":"Horiuchi M, Priola SA, Chabry J, Caughey B (2000) Interactions between heterologous forms of prion protein: binding, inhibition of conversion, and species barriers. Proc Natl Acad Sci USA 97:5836\u20135841","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR44","doi-asserted-by":"crossref","first-page":"2565","DOI":"10.1099\/0022-1317-81-10-2565","volume":"81","author":"V Iniguez","year":"2000","unstructured":"Iniguez V, McKenzie D, Mirwald J, Aiken J (2000) Strain-specific propagation of PrP(Sc) properties into baculovirus-expressed hamster PrP(C). J Gen Virol 81:2565\u20132571","journal-title":"J Gen Virol"},{"key":"708_CR45","doi-asserted-by":"crossref","first-page":"1186","DOI":"10.1136\/bmj.38804.511644.55","volume":"332","author":"JW Ironside","year":"2006","unstructured":"Ironside JW, Bishop MT, Connolly K et al (2006) Variant Creutzfeldt\u2013Jakob disease: prion protein genotype analysis of positive appendix tissue samples from a retrospective prevalence study. Br Med J 332:1186\u20131188","journal-title":"Br Med J"},{"key":"708_CR46","doi-asserted-by":"crossref","unstructured":"Ironside JW, Ghetti B, Head MW, Piccardo P, Will RG (2008) Prion diseases. In: Love S, Louis DN, Ellison DW (eds) Hodder and Arnold, pp 1197\u20131273","DOI":"10.1201\/b13319-17"},{"key":"708_CR47","doi-asserted-by":"crossref","first-page":"565","DOI":"10.1111\/j.1365-2990.2005.00697.x","volume":"31","author":"JW Ironside","year":"2005","unstructured":"Ironside JW, Ritchie DL, Head MW (2005) Phenotypic variability in human prion diseases. Neuropathol Appl Neurobiol 31:565\u2013579","journal-title":"Neuropathol Appl Neurobiol"},{"key":"708_CR48","doi-asserted-by":"crossref","first-page":"1935","DOI":"10.1126\/science.283.5409.1935","volume":"283","author":"GS Jackson","year":"1999","unstructured":"Jackson GS, Hosszu LL, Power A et al (1999) Reversible conversion of monomeric human prion protein between native and fibrilogenic conformations. Science 283:1935\u20131937","journal-title":"Science"},{"key":"708_CR49","doi-asserted-by":"crossref","first-page":"21","DOI":"10.1002\/path.2204","volume":"213","author":"M Jones","year":"2007","unstructured":"Jones M, Peden A, Prowse C et al (2007) In vitro amplification and detection of variant Creutzfeldt\u2013Jakob disease PrP(Sc). J Pathol 213:21\u201326","journal-title":"J Pathol"},{"key":"708_CR50","doi-asserted-by":"crossref","first-page":"1783","DOI":"10.1097\/WNR.0b013e328318edfa","volume":"19","author":"M Jones","year":"2008","unstructured":"Jones M, Peden AH, Wight D et al (2008) Effects of human PrPSc type and PRNP genotype in an in vitro conversion assay. Neuroreport 19:1783\u20131786","journal-title":"Neuroreport"},{"key":"708_CR51","doi-asserted-by":"crossref","first-page":"376","DOI":"10.1111\/j.1537-2995.2008.01954.x","volume":"49","author":"M Jones","year":"2008","unstructured":"Jones M, Peden AH, Yull H et al (2008) Human platelets as a substrate source for the in vitro amplification of the abnormal prion protein (PrP) associated with variant Creutzfeldt\u2013Jakob disease. Transfusion 49:376\u2013384","journal-title":"Transfusion"},{"key":"708_CR52","doi-asserted-by":"crossref","first-page":"2013","DOI":"10.3201\/eid1512.090194","volume":"15","author":"M Jones","year":"2009","unstructured":"Jones M, Wight D, Barron R et al (2009) Molecular model of prion transmission to humans. Emerg Infect Dis 15:2013\u20132016","journal-title":"Emerg Infect Dis"},{"key":"708_CR53","doi-asserted-by":"crossref","first-page":"997","DOI":"10.1006\/jmbi.1999.3386","volume":"295","author":"K Kaneko","year":"2000","unstructured":"Kaneko K, Ball HL, Wille H et al (2000) A synthetic peptide initiates Gerstmann\u2013Straussler\u2013Scheinker (GSS) disease in transgenic mice. J Mol Biol 295:997\u20131007","journal-title":"J Mol Biol"},{"key":"708_CR54","doi-asserted-by":"crossref","first-page":"2128","DOI":"10.1016\/S0140-6736(09)61568-3","volume":"374","author":"D Kaski","year":"2009","unstructured":"Kaski D, Mead S, Hyare H et al (2009) Variant CJD in an individual heterozygous for PRNP codon 129. Lancet 374:2128","journal-title":"Lancet"},{"key":"708_CR55","doi-asserted-by":"crossref","first-page":"14083","DOI":"10.1074\/jbc.C110.113464","volume":"285","author":"JI Kim","year":"2010","unstructured":"Kim JI, Cali I, Surewicz K et al (2010) Mammalian prions generated from bacterially expressed prion protein in the absence of any mammalian cofactors. J Biol Chem 285:14083\u201314087","journal-title":"J Biol Chem"},{"key":"708_CR56","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1099\/vir.0.18903-0","volume":"84","author":"L Kirby","year":"2003","unstructured":"Kirby L, Birkett CR, Rudyk H, Gilbert IH, Hope J (2003) In vitro cell-free conversion of bacterial recombinant PrP to PrPres as a model for conversion. J Gen Virol 84:1013\u20131020","journal-title":"J Gen Virol"},{"key":"708_CR57","doi-asserted-by":"crossref","first-page":"11666","DOI":"10.1073\/pnas.1834432100","volume":"100","author":"PC Klohn","year":"2003","unstructured":"Klohn PC, Stoltze L, Flechsig E, Enari M, Weissmann C (2003) A quantitative, highly sensitive cell-based infectivity assay for mouse scrapie prions. Proc Natl Acad Sci USA 100:11666\u201311671","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR58","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1038\/370471a0","volume":"370","author":"DA Kocisko","year":"1994","unstructured":"Kocisko DA, Come JH, Priola SA et al (1994) Cell-free formation of protease-resistant prion protein. Nature 370:471\u2013474","journal-title":"Nature"},{"key":"708_CR59","doi-asserted-by":"crossref","first-page":"3923","DOI":"10.1073\/pnas.92.9.3923","volume":"92","author":"DA Kocisko","year":"1995","unstructured":"Kocisko DA, Priola SA, Raymond GJ et al (1995) Species specificity in the cell-free conversion of prion protein to protease-resistant forms: a model for the scrapie species barrier. Proc Natl Acad Sci USA 92:3923\u20133927","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR60","doi-asserted-by":"crossref","first-page":"3697","DOI":"10.1128\/JVI.02561-07","volume":"82","author":"Q Kong","year":"2008","unstructured":"Kong Q, Zheng M, Casalone C et al (2008) Evaluation of the human transmission risk of an atypical bovine spongiform encephalopathy prion strain. J Virol 82:3697\u20133701","journal-title":"J Virol"},{"key":"708_CR61","doi-asserted-by":"crossref","first-page":"2649","DOI":"10.1111\/j.1460-9568.2007.05518.x","volume":"25","author":"T Kuczius","year":"2007","unstructured":"Kuczius T, Koch R, Keyvani K et al (2007) Regional and phenotype heterogeneity of cellular prion proteins in the human brain. Eur J Neurosci 25:2649\u20132655","journal-title":"Eur J Neurosci"},{"key":"708_CR62","doi-asserted-by":"crossref","first-page":"9605","DOI":"10.1128\/JVI.00635-07","volume":"81","author":"TD Kurt","year":"2007","unstructured":"Kurt TD, Perrott MR, Wilusz CJ et al (2007) Efficient in vitro amplification of chronic wasting disease PrPRES. J Virol 81:9605\u20139608","journal-title":"J Virol"},{"key":"708_CR63","doi-asserted-by":"crossref","first-page":"673","DOI":"10.1126\/science.1100195","volume":"305","author":"G Legname","year":"2004","unstructured":"Legname G, Baskakov IV, Nguyen HO et al (2004) Synthetic mammalian prions. Science 305:673\u2013676","journal-title":"Science"},{"key":"708_CR64","doi-asserted-by":"crossref","first-page":"15988","DOI":"10.1074\/jbc.M800562200","volume":"283","author":"N Makarava","year":"2008","unstructured":"Makarava N, Baskakov IV (2008) The same primary structure of the prion protein yields two distinct self-propagating states. J Biol Chem 283:15988\u201315996","journal-title":"J Biol Chem"},{"key":"708_CR65","doi-asserted-by":"crossref","first-page":"177","DOI":"10.1007\/s00401-009-0633-x","volume":"119","author":"N Makarava","year":"2010","unstructured":"Makarava N, Kovacs GG, Bocharova O et al (2010) Recombinant prion protein induces a new transmissible prion disease in wild-type animals. Acta Neuropathol 119:177\u2013187","journal-title":"Acta Neuropathol"},{"key":"708_CR66","doi-asserted-by":"crossref","first-page":"14386","DOI":"10.1074\/jbc.M900533200","volume":"284","author":"N Makarava","year":"2009","unstructured":"Makarava N, Ostapchenko VG, Savtchenko R, Baskakov IV (2009) Conformational switching within individual amyloid fibrils. J Biol Chem 284:14386\u201314395","journal-title":"J Biol Chem"},{"key":"708_CR67","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1126\/science.1090187","volume":"302","author":"G Mallucci","year":"2003","unstructured":"Mallucci G, Dickinson A, Linehan J et al (2003) Depleting neuronal PrP in prion infection prevents disease and reverses spongiosis. Science 302:871\u2013874","journal-title":"Science"},{"key":"708_CR68","doi-asserted-by":"crossref","first-page":"325","DOI":"10.1016\/j.neuron.2007.01.005","volume":"53","author":"GR Mallucci","year":"2007","unstructured":"Mallucci GR, White MD, Farmer M et al (2007) Targeting cellular prion protein reverses early cognitive deficits and neurophysiological dysfunction in prion-infected mice. Neuron 53:325\u2013335","journal-title":"Neuron"},{"key":"708_CR69","doi-asserted-by":"crossref","first-page":"1155","DOI":"10.1042\/BST0341155","volume":"34","author":"JC Manson","year":"2006","unstructured":"Manson JC, Cancellotti E, Hart P, Bishop MT, Barron RM (2006) The transmissible spongiform encephalopathies: emerging and declining epidemics. Biochem Soc Trans 34:1155\u20131158","journal-title":"Biochem Soc Trans"},{"key":"708_CR70","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1016\/j.bbrc.2009.07.163","volume":"388","author":"CE Mays","year":"2009","unstructured":"Mays CE, Titlow W, Seward T, Telling GC, Ryou C (2009) Enhancement of protein misfolding cyclic amplification by using concentrated cellular prion protein source. Biochem Biophys Res Commun 388:306\u2013310","journal-title":"Biochem Biophys Res Commun"},{"key":"708_CR71","doi-asserted-by":"crossref","first-page":"2890","DOI":"10.1099\/vir.0.82786-0","volume":"88","author":"Y Murayama","year":"2007","unstructured":"Murayama Y, Yoshioka M, Okada H et al (2007) Urinary excretion and blood level of prions in scrapie-infected hamsters. J Gen Virol 88:2890\u20132898","journal-title":"J Gen Virol"},{"key":"708_CR72","doi-asserted-by":"crossref","first-page":"153","DOI":"10.1016\/S0022-2836(02)00750-7","volume":"322","author":"PK Nandi","year":"2002","unstructured":"Nandi PK, Leclerc E, Nicole JC, Takahashi M (2002) DNA-induced partial unfolding of prion protein leads to its polymerisation to amyloid. J Mol Biol 322:153\u2013161","journal-title":"J Mol Biol"},{"key":"708_CR73","doi-asserted-by":"crossref","first-page":"14129","DOI":"10.1021\/bi061526k","volume":"45","author":"KA Nishina","year":"2006","unstructured":"Nishina KA, Deleault NR, Mahal SP et al (2006) The stoichiometry of host PrPC glycoforms modulates the efficiency of PrPSc formation in vitro. Biochemistry 45:14129\u201314139","journal-title":"Biochemistry"},{"key":"708_CR74","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1093\/protein\/gzp031","volume":"22","author":"CD Orru","year":"2009","unstructured":"Orru CD, Wilham JM, Hughson AG et al (2009) Human variant Creutzfeldt\u2013Jakob disease and sheep scrapie PrP(res) detection using seeded conversion of recombinant prion protein. Protein Eng Des Sel 22:515\u2013521","journal-title":"Protein Eng Des Sel"},{"key":"708_CR75","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 et al (1999) Classification of sporadic Creutzfeldt\u2013Jakob disease based on molecular and phenotypic analysis of 300 subjects. Ann Neurol 46:224\u2013233","journal-title":"Ann Neurol"},{"key":"708_CR76","doi-asserted-by":"crossref","unstructured":"Peden A, McCardle L, Head MW et al. (2010) Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia. Haemophilia","DOI":"10.1111\/j.1365-2516.2009.02181.x"},{"key":"708_CR77","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/S0140-6736(04)16811-6","volume":"364","author":"AH Peden","year":"2004","unstructured":"Peden AH, Head MW, Ritchie DL, Bell JE, Ironside JW (2004) Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient. Lancet 364:527\u2013529","journal-title":"Lancet"},{"key":"708_CR78","doi-asserted-by":"crossref","first-page":"1307","DOI":"10.1515\/bchm.1998.379.11.1307","volume":"379","author":"K Post","year":"1998","unstructured":"Post K, Pitschke M, Schafer O et al (1998) Rapid acquisition of beta-sheet structure in the prion protein prior to multimer formation. Biol Chem 379:1307\u20131317","journal-title":"Biol Chem"},{"key":"708_CR79","doi-asserted-by":"crossref","first-page":"6692","DOI":"10.1093\/emboj\/20.23.6692","volume":"20","author":"SA Priola","year":"2001","unstructured":"Priola SA, Lawson VA (2001) Glycosylation influences cross-species formation of protease-resistant prion protein. EMBO J 20:6692\u20136699","journal-title":"EMBO J"},{"key":"708_CR80","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 USA 95:13363\u201313383","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR81","doi-asserted-by":"crossref","first-page":"19121","DOI":"10.1074\/jbc.275.25.19121","volume":"275","author":"K Qin","year":"2000","unstructured":"Qin K, Yang DS, Yang Y et al (2000) Copper(II)-induced conformational changes and protease resistance in recombinant and cellular PrP. Effect of protein age and deamidation. J Biol Chem 275:19121\u201319131","journal-title":"J Biol Chem"},{"key":"708_CR82","doi-asserted-by":"crossref","first-page":"285","DOI":"10.1038\/40876","volume":"388","author":"GJ Raymond","year":"1997","unstructured":"Raymond GJ, Hope J, Kocisko DA et al (1997) Molecular assessment of the potential transmissibilities of BSE and scrapie to humans. Nature 388:285\u2013288","journal-title":"Nature"},{"key":"708_CR83","doi-asserted-by":"crossref","first-page":"35245","DOI":"10.1074\/jbc.M603964200","volume":"281","author":"P Saa","year":"2006","unstructured":"Saa P, Castilla J, Soto C (2006) Ultra-efficient replication of infectious prions by automated protein misfolding cyclic amplification. J Biol Chem 281:35245\u201335252","journal-title":"J Biol Chem"},{"key":"708_CR84","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1038\/35081095","volume":"411","author":"GP Saborio","year":"2001","unstructured":"Saborio GP, Permanne B, Soto C (2001) Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding. Nature 411:810\u2013813","journal-title":"Nature"},{"key":"708_CR85","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1006\/bbrc.1999.0660","volume":"258","author":"GP Saborio","year":"1999","unstructured":"Saborio GP, Soto C, Kascsak RJ et al (1999) Cell-lysate conversion of prion protein into its protease-resistant isoform suggests the participation of a cellular chaperone. Biochem Biophys Res Commun 258:470\u2013475","journal-title":"Biochem Biophys Res Commun"},{"key":"708_CR86","doi-asserted-by":"crossref","first-page":"213","DOI":"10.1016\/j.jbbm.2005.05.004","volume":"63","author":"NI Sarafoff","year":"2005","unstructured":"Sarafoff NI, Bieschke J, Giese A et al (2005) Automated PrPres amplification using indirect sonication. J Biochem Biophys Methods 63:213\u2013221","journal-title":"J Biochem Biophys Methods"},{"key":"708_CR87","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1073\/pnas.0810680105","volume":"106","author":"CJ Sigurdson","year":"2009","unstructured":"Sigurdson CJ, Nilsson KP, Hornemann S et al (2009) De novo generation of a transmissible spongiform encephalopathy by mouse transgenesis. Proc Natl Acad Sci USA 106:304\u2013309","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR88","doi-asserted-by":"crossref","first-page":"638","DOI":"10.1016\/j.febslet.2004.12.035","volume":"579","author":"C Soto","year":"2005","unstructured":"Soto C, Anderes L, Suardi S et al (2005) Pre-symptomatic detection of prions by cyclic amplification of protein misfolding. FEBS Lett 579:638\u2013642","journal-title":"FEBS Lett"},{"key":"708_CR89","doi-asserted-by":"crossref","first-page":"996","DOI":"10.1111\/j.1537-2995.2010.02595.x","volume":"50","author":"MH Tattum","year":"2010","unstructured":"Tattum MH, Jones S, Pal S, Collinge J, Jackson GS (2010) Discrimination between prion-infected and normal blood samples by protein misfolding cyclic amplification. Transfusion 50:996\u20131002","journal-title":"Transfusion"},{"key":"708_CR90","doi-asserted-by":"crossref","first-page":"3177","DOI":"10.1099\/vir.0.2008\/004226-0","volume":"89","author":"L Thorne","year":"2008","unstructured":"Thorne L, Terry LA (2008) In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie. J Gen Virol 89:3177\u20133184","journal-title":"J Gen Virol"},{"key":"708_CR91","doi-asserted-by":"crossref","first-page":"e1000029","DOI":"10.1371\/journal.ppat.1000029","volume":"4","author":"E Uro-Coste","year":"2008","unstructured":"Uro-Coste E, Cassard H, Simon S et al (2008) Beyond PrP res type 1\/type 2 dichotomy in Creutzfeldt\u2013Jakob disease. PLoS Pathog 4:e1000029","journal-title":"PLoS Pathog"},{"key":"708_CR92","doi-asserted-by":"crossref","first-page":"1132","DOI":"10.1126\/science.1183748","volume":"327","author":"F Wang","year":"2010","unstructured":"Wang F, Wang X, Yuan CG, Ma J (2010) Generating a prion with bacterially expressed recombinant prion protein. Science 327:1132\u20131135","journal-title":"Science"},{"key":"708_CR93","doi-asserted-by":"crossref","first-page":"826","DOI":"10.1177\/104063870902100609","volume":"21","author":"PR Webb","year":"2009","unstructured":"Webb PR, Powell L, Denyer M et al (2009) A retrospective immunohistochemical study reveals atypical scrapie has existed in the United Kingdom since at least 1987. J Vet Diagn Invest 21:826\u2013829","journal-title":"J Vet Diagn Invest"},{"key":"708_CR94","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1098\/rstb.1994.0040","volume":"343","author":"C Weissmann","year":"1994","unstructured":"Weissmann C, Bueler H, Fischer M, Sauer A, Aguet M (1994) Susceptibility to scrapie in mice is dependent on PrPC. Philos Trans R Soc Lond B Biol Sci 343:431\u2013433","journal-title":"Philos Trans R Soc Lond B Biol Sci"},{"key":"708_CR95","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1016\/j.bbadis.2007.02.011","volume":"1772","author":"L Westergard","year":"2007","unstructured":"Westergard L, Christensen HM, Harris DA (2007) The cellular prion protein (PrP(C)): its physiological function and role in disease. Biochim Biophys Acta 1772:629\u2013644","journal-title":"Biochim Biophys Acta"},{"key":"708_CR96","doi-asserted-by":"crossref","first-page":"10238","DOI":"10.1073\/pnas.0802759105","volume":"105","author":"MD White","year":"2008","unstructured":"White MD, Farmer M, Mirabile I et al (2008) Single treatment with RNAi against prion protein rescues early neuronal dysfunction and prolongs survival in mice with prion disease. Proc Natl Acad Sci USA 105:10238\u201310243","journal-title":"Proc Natl Acad Sci USA"},{"key":"708_CR97","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 et al (1996) A new variant of Creutzfeldt\u2013Jakob disease in the UK. Lancet 347:921\u2013925","journal-title":"Lancet"},{"key":"708_CR98","doi-asserted-by":"crossref","first-page":"669","DOI":"10.1046\/j.1471-4159.2001.00606.x","volume":"79","author":"LW Xiong","year":"2001","unstructured":"Xiong LW, Raymond LD, Hayes SF, Raymond GJ, Caughey B (2001) Conformational change, aggregation and fibril formation induced by detergent treatments of cellular prion protein. J Neurochem 79:669\u2013678","journal-title":"J Neurochem"},{"key":"708_CR99","doi-asserted-by":"crossref","first-page":"43942","DOI":"10.1074\/jbc.M203611200","volume":"277","author":"WQ Zou","year":"2002","unstructured":"Zou WQ, Cashman NR (2002) Acidic pH and detergents enhance in vitro conversion of human brain PrPC to a PrPSc-like form. J Biol Chem 277:43942\u201343947","journal-title":"J Biol Chem"}],"container-title":["Acta Neuropathologica"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-010-0708-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s00401-010-0708-8\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s00401-010-0708-8","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,6,5]],"date-time":"2020-06-05T14:17:09Z","timestamp":1591366629000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s00401-010-0708-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,6,10]]},"references-count":99,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2011,1]]}},"alternative-id":["708"],"URL":"https:\/\/doi.org\/10.1007\/s00401-010-0708-8","relation":{},"ISSN":["0001-6322","1432-0533"],"issn-type":[{"value":"0001-6322","type":"print"},{"value":"1432-0533","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,6,10]]}}}