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We assessed PMCA of pathological prion protein (PrP<jats:sup>TSE<\/jats:sup>) from 14 human sCJD brain samples in 3 substrates: 2 from transgenic mice expressing human prion protein (PrP) with either methionine (M) or valine (V) at position 129, and 1 from bank voles. Brain extracts representing the 5 major clinicopathological sCJD subtypes (MM1\/MV1, MM2, MV2, VV1, and VV2) all triggered seeded PrP<jats:sup>TSE<\/jats:sup> amplification during serial PMCA with strong seed- and substrate-dependence. Remarkably, bank vole PrP substrate allowed the propagation of all sCJD subtypes with preservation of the initial molecular PrP<jats:sup>TSE<\/jats:sup> type. In contrast, PMCA in human PrP substrates was accompanied by a PrP<jats:sup>TSE<\/jats:sup> molecular shift during heterologous (M\/V129) PMCA reactions, with increased permissiveness of V129 PrP substrate to in vitro sCJD prion amplification compared to M129 PrP substrate. Combining PMCA amplification sensitivities with PrP<jats:sup>TSE<\/jats:sup> electrophoretic profiles obtained in the different substrates confirmed the classification of 4 distinct major sCJD prion strains (M1, M2, V1, and V2). Finally, the level of sensitivity required to detect VV2 sCJD prions in cerebrospinal fluid was achieved.<\/jats:p>","DOI":"10.1038\/s41598-021-83630-1","type":"journal-article","created":{"date-parts":[[2021,2,20]],"date-time":"2021-02-20T09:14:59Z","timestamp":1613812499000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Sensitive protein misfolding cyclic amplification of sporadic Creutzfeldt\u2013Jakob disease prions is strongly seed and substrate dependent"],"prefix":"10.1038","volume":"11","author":[{"given":"Maxime","family":"B\u00e9londrade","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Simon","family":"Nicot","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Charly","family":"Mayran","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lilian","family":"Bruyere-Ostells","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Florian","family":"Almela","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michele A.","family":"Di Bari","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Etienne","family":"Levavasseur","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joel C.","family":"Watts","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chantal","family":"Fournier-Wirth","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sylvain","family":"Lehmann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"St\u00e9phane","family":"Ha\u00efk","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Romolo","family":"Nonno","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daisy","family":"Bougard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2021,2,18]]},"reference":[{"key":"83630_CR1","doi-asserted-by":"publisher","first-page":"574","DOI":"10.1111\/nan.12461","volume":"44","author":"A Franceschini","year":"2018","unstructured":"Franceschini, A., Strammiello, R., Capellari, S., Giese, A. & Parchi, P. 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