{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,30]],"date-time":"2026-10-30T09:55:52Z","timestamp":1793354152535,"version":"4.5.0"},"reference-count":55,"publisher":"Public Library of Science (PLoS)","issue":"7","license":[{"start":{"date-parts":[[2013,7,3]],"date-time":"2013-07-03T00:00:00Z","timestamp":1372809600000},"content-version":"unspecified","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["www.plosone.org"],"crossmark-restriction":false},"short-container-title":["PLoS ONE"],"DOI":"10.1371\/journal.pone.0066352","type":"journal-article","created":{"date-parts":[[2014,4,30]],"date-time":"2014-04-30T16:18:35Z","timestamp":1398874715000},"page":"e66352","update-policy":"https:\/\/doi.org\/10.1371\/journal.pone.corrections_policy","source":"Crossref","is-referenced-by-count":42,"title":["Re-Assessment of PrPSc Distribution in Sporadic and Variant CJD"],"prefix":"10.1371","volume":"8","author":[{"given":"Richard","family":"Rubenstein","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Binggong","family":"Chang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"340","published-online":{"date-parts":[[2013,7,3]]},"reference":[{"key":"ref1","doi-asserted-by":"crossref","first-page":"10962","DOI":"10.1073\/pnas.90.23.10962","article-title":"Conversion of a-helices into bsheets features in the formation of the scrapie prion proteins","volume":"90","author":"KM Pan","year":"1993","journal-title":"Proc Natl Acad Sci USA"},{"key":"ref2","doi-asserted-by":"crossref","first-page":"171","DOI":"10.1016\/S0140-6736(01)05403-4","article-title":"Tissue distribution of protease resistant prion protein in variant Creutzfeldt\u2013Jakob disease using a highly sensitive immunoblotting assay","volume":"358","author":"JDF Wadsworth","year":"2001","journal-title":"Lancet"},{"key":"ref3","doi-asserted-by":"crossref","first-page":"1812","DOI":"10.1056\/NEJMoa030351","article-title":"Extraneural pathologic prion protein in sporadic Creutzfeldt-Jakob disease","volume":"349","author":"M Glatzel","year":"2003","journal-title":"N Engl J Med"},{"key":"ref4","doi-asserted-by":"crossref","first-page":"214","DOI":"10.1016\/S1473-3099(03)00578-4","article-title":"Organ distribution of prion proteins in variant Creutzfeldt\u2013Jakob disease","volume":"3","author":"I Ramasamy","year":"2003","journal-title":"Lancet Infect Dis"},{"key":"ref5","doi-asserted-by":"crossref","first-page":"185","DOI":"10.1016\/0092-8674(90)90225-4","article-title":"Cellular isoform of the scrapie agent protein participates in lymphocyte activation","volume":"61","author":"NR Cashman","year":"1990","journal-title":"Cell"},{"key":"ref6","doi-asserted-by":"crossref","first-page":"1935","DOI":"10.1099\/0022-1317-77-8-1935","article-title":"Replication of scrapie in spleens of SCID mice follows reconstitution with wild-type mouse bone marrow","volume":"77","author":"H Fraser","year":"1996","journal-title":"J Gen Virol"},{"key":"ref7","doi-asserted-by":"crossref","first-page":"69","DOI":"10.1038\/37981","article-title":"PrP-expressing tissue required for transfer of scrapie infectivity from spleen to brain","volume":"389","author":"T Bl\u00e4ttler","year":"1997","journal-title":"Nature"},{"key":"ref8","doi-asserted-by":"crossref","first-page":"1308","DOI":"10.1038\/15264","article-title":"Scrapie replication in lymphoid tissues depends on prion protein-expressing follicular dendritic cells","volume":"5","author":"KL Brown","year":"1999","journal-title":"Nat Med"},{"key":"ref9","doi-asserted-by":"crossref","first-page":"63","DOI":"10.1111\/j.1423-0410.2005.00683.x","article-title":"Blood infectivity, processing and screening tests in transmissible spongiform encephalopathy","volume":"89","author":"P Brown","year":"2005","journal-title":"Vox Sang"},{"key":"ref10","doi-asserted-by":"crossref","first-page":"1169","DOI":"10.1046\/j.1537-2995.1999.39111169.x","article-title":"Further studies of blood infectivity in an experimental model of transmissible spongiform encephalopathy, with an explanation of why blood components do not transmit Creutzfeldt\u2013Jakob disease in humans","volume":"39","author":"P Brown","year":"1999","journal-title":"Transfusion"},{"key":"ref11","doi-asserted-by":"crossref","first-page":"1687","DOI":"10.1046\/j.0041-1132.2003.00586.x","article-title":"Similar levels of infectivity in the blood of mice infected with human-derived vCJD and GSS strains of transmissible spongiform encephalopathy","volume":"43","author":"L Cervenakova","year":"2003","journal-title":"Transfusion"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"417","DOI":"10.1016\/S0140-6736(04)15486-X","article-title":"Possible transmission of variant Creutzfeldt\u2013Jakob disease by blood transfusion","volume":"363","author":"CA Llewelyn","year":"2004","journal-title":"Lancet"},{"key":"ref13","doi-asserted-by":"crossref","first-page":"527","DOI":"10.1016\/S0140-6736(04)16811-6","article-title":"Preclinical vCJD after blood transfusion in a PRNP codon 129 heterozygous patient","volume":"364","author":"AH Peden","year":"2004","journal-title":"Lancet"},{"key":"ref14","doi-asserted-by":"crossref","first-page":"2061","DOI":"10.1016\/S0140-6736(06)69835-8","article-title":"Clinical presentation and pre-mortem diagnosis of variant Creutzfeldt\u2013Jakob disease associated with blood transfusion: a case report","volume":"368","author":"SJ Wroe","year":"2006","journal-title":"Lancet"},{"key":"ref15","doi-asserted-by":"crossref","first-page":"744","DOI":"10.1136\/jnnp.70.6.744","article-title":"Use of 14-3-3 and other brain-specific proteins in CSF in the diagnosis of variant Creutzfeldt-Jakob disease","volume":"70","author":"AJ Green","year":"2001","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref16","doi-asserted-by":"crossref","first-page":"89","DOI":"10.1136\/jnnp.2005.065755","article-title":"Raised CSF phospho-tau concentrations in variant Creutzfeldt-Jakob disease: diagnostic and pathological implications","volume":"77","author":"CA Goodall","year":"2006","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref17","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1186\/1471-2377-11-133","article-title":"Diagnostic accuracy of cerebrospinal fluid protein markers for sporadic Creutzfeldt-Jakob disease in Canada: a 6-year prospective study","volume":"11","author":"MB Coulthart","year":"2011","journal-title":"BMC Neurology"},{"key":"ref18","doi-asserted-by":"crossref","first-page":"175","DOI":"10.1038\/nm.2294","article-title":"Ultrasensitive human prion detection in cerebrospinal fluid by real-time quaking-induced conversion","volume":"17","author":"R Atarashi","year":"2011","journal-title":"Nat Med"},{"key":"ref19","doi-asserted-by":"crossref","first-page":"278","DOI":"10.1002\/ana.23589","article-title":"Real Time Quaking-Induced Conversion Analysis of Cerebrospinal Fluid in Sporadic Creutzfeldt\u2013Jakob Disease","volume":"72","author":"LI McGuire","year":"2012","journal-title":"Ann Neurol"},{"key":"ref20","doi-asserted-by":"crossref","first-page":"810","DOI":"10.1038\/35081095","article-title":"Sensitive detection of pathological prion protein by cyclic amplification of protein misfolding","volume":"411","author":"GP Saborio","year":"2001","journal-title":"Nature"},{"key":"ref21","doi-asserted-by":"crossref","first-page":"1883","DOI":"10.1099\/vir.0.020164-0","article-title":"A novel method for preclinical detection of PrPSc in blood","volume":"91","author":"R Rubenstein","year":"2010","journal-title":"J Gen Virol"},{"key":"ref22","doi-asserted-by":"crossref","first-page":"9031","DOI":"10.1128\/JVI.05111-11","article-title":"Prion Disease Detection, PMCA Kinetics, and IgG in Urine from Sheep Naturally\/Experimentally Infected with Scrapie and Deer with Preclinical\/Clinical Chronic Wasting Disease","volume":"85","author":"R Rubenstein","year":"2011","journal-title":"J Virol"},{"key":"ref23","doi-asserted-by":"crossref","first-page":"183","DOI":"10.1016\/S0140-6736(98)12075-5","article-title":"Investigation of variant Creutzfeldt-Jakob disease and other human prion diseases with tonsil biopsy samples","volume":"353","author":"AF Hill","year":"1999","journal-title":"Lancet"},{"key":"ref24","doi-asserted-by":"crossref","first-page":"1375","DOI":"10.1099\/vir.0.038802-0","article-title":"PrPSc detection and infectivity in semen from scrapie-infected sheep","volume":"93","author":"R Rubenstein","year":"2012","journal-title":"J Gen Virol"},{"key":"ref25","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1016\/j.jviromet.2009.02.019","article-title":"Surround optical fiber immunoassay (SOFIA): An ultra-sensitive assay for prion protein detection","volume":"159","author":"B Chang","year":"2009","journal-title":"J Virol Meth"},{"key":"ref26","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1016\/S0002-9440(10)63105-7","article-title":"Peripheral Tissue Involvement in Sporadic, Iatrogenic, and Variant Creutzfeldt-Jakob Disease An Immunohistochemical, Quantitative, and Biochemical Study","volume":"164","author":"MW Head","year":"2004","journal-title":"Am J Path"},{"key":"ref27","doi-asserted-by":"crossref","first-page":"1157","DOI":"10.1038\/2654","article-title":"Eight prion strains have PrP(Sc) molecules with different conformations","volume":"4","author":"J Safar","year":"1998","journal-title":"Nat Med"},{"key":"ref28","doi-asserted-by":"crossref","first-page":"14339","DOI":"10.1128\/JVI.79.22.14339-14345.2005","article-title":"PrPTSE Distribution in a Primate Model of Variant, Sporadic, and Iatrogenic Creutzfeldt-Jakob Disease","volume":"79","author":"C Herzog","year":"2005","journal-title":"J Virol"},{"key":"ref29","unstructured":"World Health Organization (2006) Guidelines on Tissue Infectivity Distribution in Transmissible Spongiform Encephalopathies. Geneva, Sept. 14\u201315, 2005."},{"key":"ref30","doi-asserted-by":"crossref","first-page":"3630","DOI":"10.1002\/jcb.23286","article-title":"High CJD infectivity remains after prion protein is destroyed","volume":"112","author":"K Miyazawa","year":"2011","journal-title":"J Cell Biochem"},{"key":"ref31","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1136\/jnnp.59.4.349","article-title":"Cerebrospinal fluid","volume":"59","author":"EJ Thompson","year":"1995","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"ref32","first-page":"3","article-title":"Diseases, blood and the immune system: concerns and reality","volume":"85","author":"A Aguzzi","year":"2000","journal-title":"Haematologica"},{"key":"ref33","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/S0167-5699(98)01253-5","article-title":"Involvement of the immune system in TSE pathogenesis","volume":"19","author":"NA Mabbott","year":"1998","journal-title":"Immunol Today"},{"key":"ref34","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/nmeth0308-211","article-title":"Simlified ultrasensitive prion detection by recombinant PrP conversion with shaking. 2008","volume":"5","author":"R Atarashi","year":"2008","journal-title":"Nat Methods"},{"key":"ref35","doi-asserted-by":"crossref","first-page":"515","DOI":"10.1093\/protein\/gzp031","article-title":"Human variant Creutzfeldt\u2013Jakob disease and sheep scrapie PrP<sup>res<\/sup> detection using seeded conversion of recombinant prion protein","volume":"22","author":"CD Orr\u00fa","year":"2009","journal-title":"Protein Eng Des Sel"},{"key":"ref36","doi-asserted-by":"crossref","first-page":"3177","DOI":"10.1099\/vir.0.2008\/004226-0","article-title":"In vitro amplification of PrPSc derived from the brain and blood of sheep infected with scrapie","volume":"89","author":"L Thorne","year":"2008","journal-title":"J Gen Virol"},{"key":"ref37","doi-asserted-by":"crossref","first-page":"105","DOI":"10.1007\/BF01309373","article-title":"Sustained viremia in experimental hamster scrapie","volume":"82","author":"H Diringer","year":"1984","journal-title":"Arch Virol"},{"key":"ref38","doi-asserted-by":"crossref","first-page":"145","DOI":"10.1007\/BF01313752","article-title":"Levels of infectivity in the blood throughout the incubation period of hamsters peripherally injected with scrapie","volume":"108","author":"P Casaccia","year":"1989","journal-title":"Arch Virol"},{"key":"ref39","doi-asserted-by":"crossref","first-page":"896","DOI":"10.1016\/S0140-6736(85)90165-5","article-title":"Transmission to animals of Creutzfeldt-Jakob disease from human blood","volume":"2","author":"EE Manuelidis","year":"1985","journal-title":"Lancet"},{"key":"ref40","doi-asserted-by":"crossref","first-page":"1074","DOI":"10.1016\/S0140-6736(85)90949-3","article-title":"Transmission of Creutzfeldt-Jakob disease from human blood and urine into mice","volume":"2","author":"J Tateishi","year":"1985","journal-title":"Lancet"},{"key":"ref41","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1056\/NEJM199208273270918","article-title":"Demonstration of the transmissible agent in tissue from a pregnant woman with Creutzfeldt-Jakob disease","volume":"327","author":"Y Tamai","year":"1992","journal-title":"N Engl J Med"},{"key":"ref42","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1016\/S0140-6736(94)92046-X","article-title":"Selection of specific strains in iatrogenic Creutzfeldt-Jakob disease","volume":"343","author":"JP Deslys","year":"1994","journal-title":"Lancet"},{"key":"ref43","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1067\/mlc.2001.111951","article-title":"Blood infectivity and the prospects for a diagnostic screening test in Creutzfeldt-Jakob disease","volume":"137","author":"P Brown","year":"2001","journal-title":"J Lab Clin Med 2001"},{"key":"ref44","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1002\/ana.410350504","article-title":"Human spongiform encephalopathy: the National Institutes of Health series of 300 cases of experimentally transmitted disease","volume":"35","author":"P Brown","year":"1994","journal-title":"Ann Neurol"},{"key":"ref45","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/j.cell.2005.02.011","article-title":"In vitro generation of infectious scrapie prions","volume":"121","author":"J Castilla","year":"2005","journal-title":"Cell"},{"key":"ref46","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1038\/nm1286","article-title":"Detection of prions in blood","volume":"11","author":"J Castilla","year":"2005","journal-title":"Nat Med"},{"key":"ref47","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1126\/science.1129051","article-title":"Presymptomatic detection of prions in blood","volume":"313","author":"P Saa","year":"2006","journal-title":"Science"},{"key":"ref48","doi-asserted-by":"crossref","first-page":"2890","DOI":"10.1099\/vir.0.82786-0","article-title":"Urinary excretion and blood level of prions in scrapie-infected hamsters","volume":"88","author":"Y Murayama","year":"2007","journal-title":"J Gen Virol"},{"key":"ref49","doi-asserted-by":"crossref","first-page":"3161","DOI":"10.1016\/j.febslet.2008.08.003","article-title":"Detection of infectious prions in urine","volume":"582","author":"D Gonzalez-Romero","year":"2008","journal-title":"FEBS Lett"},{"key":"ref50","doi-asserted-by":"crossref","first-page":"e4848","DOI":"10.1371\/journal.pone.0004848","article-title":"Detection of CWD prions in urine and saliva of deer by transgenic mouse bioassay","volume":"4","author":"NJ Haley","year":"2009","journal-title":"PLoS One"},{"key":"ref51","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1111\/j.1423-0410.2006.00833.x","article-title":"Creutzfeldt- Jakob disease and blood transfusion: results of the UK Transfusion Medicine Epidemiological Review study","volume":"91","author":"PE Hewitt","year":"2006","journal-title":"Vox Sang"},{"key":"ref52","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1111\/j.1423-0410.2009.01161.x","article-title":"A report on transmissible spongiform encephalopathies and transfusion safety","volume":"96","author":"J Coste","year":"2009","journal-title":"Vox Sang"},{"key":"ref53","doi-asserted-by":"crossref","first-page":"1003","DOI":"10.1111\/j.1537-2995.2010.02614.x","article-title":"Variant Creutzfeldt-Jakob disease in a transfusion recipient: coincidence or cause?","volume":"50","author":"G Chohan","year":"2010","journal-title":"Transfusion"},{"key":"ref54","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1111\/j.1365-2516.2009.02181.x","article-title":"Variant CJD infection in the spleen of a neurologically asymptomatic UK adult patient with haemophilia","volume":"16","author":"A Peden","year":"2010","journal-title":"Haemophilia"},{"key":"ref55","doi-asserted-by":"crossref","first-page":"1366","DOI":"10.3201\/eid1509.090253","article-title":"Susceptibilities of Nonhuman Primates to Chronic Wasting Disease","volume":"15","author":"B Race","year":"2009","journal-title":"Emerg Infect Dis"}],"container-title":["PLoS ONE"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/dx.plos.org\/10.1371\/journal.pone.0066352","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,5,8]],"date-time":"2020-05-08T20:43:28Z","timestamp":1588970608000},"score":1,"resource":{"primary":{"URL":"https:\/\/dx.plos.org\/10.1371\/journal.pone.0066352"}},"subtitle":[],"editor":[{"given":"Thomas","family":"Wisniewski","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"editor"}]}],"short-title":[],"issued":{"date-parts":[[2013,7,3]]},"references-count":55,"journal-issue":{"issue":"7","published-online":{"date-parts":[[2013,7,3]]}},"URL":"https:\/\/doi.org\/10.1371\/journal.pone.0066352","relation":{},"ISSN":["1932-6203"],"issn-type":[{"value":"1932-6203","type":"electronic"}],"subject":[],"published":{"date-parts":[[2013,7,3]]}}}