{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,13]],"date-time":"2026-03-13T20:15:05Z","timestamp":1773432905874,"version":"3.50.1"},"reference-count":21,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2018,2,5]],"date-time":"2018-02-05T00:00:00Z","timestamp":1517788800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"funder":[{"name":"Department of Health Policy Research Programme and the Scottish Government"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["The Journal of Pathology CR"],"published-print":{"date-parts":[[2018,4]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Human prion diseases constitute a group of infectious and invariably fatal neurodegenerative disorders associated with misfolding of the prion protein. Variant Creutzfeldt\u2013Jakob disease (vCJD) is a zoonotic prion disease linked to oral exposure to the infectious agent that causes bovine spongiform encephalopathy (BSE) in cattle. The most recent case of definite vCJD was heterozygous (MV) at polymorphic codon 129 of the prion protein gene <jats:italic>PRNP<\/jats:italic> while all of the previous 177 definite or probable vCJD cases who underwent genetic analysis were methionine homozygous (MM). Retrospective prevalence studies conducted on lympho\u2010reticular tissue suggest that the number of asymptomatic vCJD carriers in the United Kingdom might be around 1 in 2000 people. In addition, there have been four known cases of the transmission of vCJD infection via blood transfusion. For these reasons, a sensitive, reliable, and fast diagnostic test is currently needed. We describe a rapid and highly sensitive seeding conversion assay that detects disease\u2010associated prion protein in the brain and cerebrospinal fluid in vCJD after 48\u201396 h of amplification, with 100% sensitivity and specificity. This method can amplify prions from definite, probable, and possible vCJD cases from patients who are either MM or MV at <jats:italic>PRNP<\/jats:italic>\u2010codon 129.<\/jats:p>","DOI":"10.1002\/cjp2.90","type":"journal-article","created":{"date-parts":[[2017,12,18]],"date-time":"2017-12-18T11:02:36Z","timestamp":1513594956000},"page":"86-92","source":"Crossref","is-referenced-by-count":34,"title":["Rapid amplification of prions from variant Creutzfeldt\u2013Jakob disease cerebrospinal fluid"],"prefix":"10.1002","volume":"4","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-4789-7401","authenticated-orcid":false,"given":"Marcelo A","family":"Barria","sequence":"first","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine The University of Edinburgh Edinburgh UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Lee","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine The University of Edinburgh Edinburgh UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alison JE","family":"Green","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine The University of Edinburgh Edinburgh UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Richard","family":"Knight","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine The University of Edinburgh Edinburgh UK"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark W","family":"Head","sequence":"additional","affiliation":[{"name":"National CJD Research &amp; Surveillance Unit, Centre for Clinical Brain Sciences, Deanery of Clinical Medicine The University of Edinburgh Edinburgh UK"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2018,2,5]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1111\/neup.12016"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1365-2990.2012.01265.x"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)91412-9"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/bmj.f5675"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.3201\/eid2001.130858"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00401-016-1638-x"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0146833"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aaf6188"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1002\/acn3.338"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.ppat.1004202"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23589"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/ana.24679"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1404401"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2016.4547"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.neulet.2011.04.072"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1016\/S1474-4422(06)70413-6"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1126\/scitranslmed.aag1257"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1074\/jbc.M116.745851"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1021\/bi200950v"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMoa1315200"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMc1610003"}],"container-title":["The Journal of Pathology: Clinical Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fcjp2.90","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/pathsocjournals.onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/cjp2.90","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,12]],"date-time":"2025-10-12T10:50:46Z","timestamp":1760266246000},"score":1,"resource":{"primary":{"URL":"https:\/\/pathsocjournals.onlinelibrary.wiley.com\/doi\/10.1002\/cjp2.90"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,2,5]]},"references-count":21,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2018,4]]}},"alternative-id":["10.1002\/cjp2.90"],"URL":"https:\/\/doi.org\/10.1002\/cjp2.90","archive":["Portico"],"relation":{},"ISSN":["2056-4538","2056-4538"],"issn-type":[{"value":"2056-4538","type":"print"},{"value":"2056-4538","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,2,5]]}}}