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Recently, PrP plaques (p) have been described in the white matter of a small group of CJD (p-CJD) cases with the 129MM genotype and carrying resPrP<jats:sup>D<\/jats:sup> type 1 (T1). Despite the different histopathological phenotype, the gel mobility and molecular features of p-CJD resPrP<jats:sup>D<\/jats:sup> T1 mimic those of sCJDMM1, the most common human prion disease. Here, we describe the clinical features, histopathology, and molecular properties of two distinct PrP plaque phenotypes affecting the gray matter (p<jats:sup>GM<\/jats:sup>) or the white matter (p<jats:sup>WM<\/jats:sup>) of sCJD cases with the PrP 129MM genotype (sCJDMM). Prevalence of p<jats:sup>GM<\/jats:sup>- and p<jats:sup>WM<\/jats:sup>-CJD proved comparable and was estimated to be\u2009~\u20090.6% among sporadic prion diseases and\u2009~\u20091.1% among the sCJDMM group. Mean age at onset (61 and 68\u00a0years) and disease duration (~\u20097\u00a0months) of p<jats:sup>WM<\/jats:sup>- and p<jats:sup>GM<\/jats:sup>-CJD did not differ significantly. PrP plaques were mostly confined to the cerebellar cortex in p<jats:sup>GM<\/jats:sup>-CJD, but were ubiquitous in p<jats:sup>WM<\/jats:sup>-CJD. Typing of resPrP<jats:sup>D<\/jats:sup> T1 showed an unglycosylated fragment of\u2009~\u200920\u00a0kDa (T1<jats:sup>20<\/jats:sup>) in p<jats:sup>GM<\/jats:sup>-CJD and sCJDMM1 patients, while a doublet of\u2009~\u200921\u201320\u00a0kDa (T1<jats:sup>21\u221220<\/jats:sup>) was a molecular signature of p<jats:sup>WM<\/jats:sup>-CJD in subcortical regions. In addition, conformational characteristics of p<jats:sup>WM<\/jats:sup>-CJD resPrP<jats:sup>D<\/jats:sup> T1 differed from those of p<jats:sup>GM<\/jats:sup>-CJD and sCJDMM1. Inoculation of p<jats:sup>WM<\/jats:sup>-CJD and sCJDMM1 brain extracts to transgenic mice expressing human PrP reproduced the histotype with PrP plaques only in mice challenged with p<jats:sup>WM<\/jats:sup>-CJD. Furthermore, T1<jats:sup>20<\/jats:sup> of p<jats:sup>WM<\/jats:sup>-CJD, but not T1<jats:sup>21<\/jats:sup>, was propagated in mice. These data suggest that T1<jats:sup>21<\/jats:sup> and T1<jats:sup>20<\/jats:sup> of p<jats:sup>WM<\/jats:sup>-CJD, and T1<jats:sup>20<\/jats:sup> of sCJDMM1 are distinct prion strains. Further studies are required to shed light on the etiology of p-CJD cases, particularly those of T1<jats:sup>20<\/jats:sup> of the novel p<jats:sup>GM<\/jats:sup>-CJD subtype.<\/jats:p>","DOI":"10.1007\/s00401-023-02581-1","type":"journal-article","created":{"date-parts":[[2023,5,8]],"date-time":"2023-05-08T17:04:44Z","timestamp":1683565484000},"page":"121-143","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":8,"title":["A novel subtype of sporadic Creutzfeldt\u2013Jakob disease with PRNP codon 129MM genotype and PrP plaques"],"prefix":"10.1007","volume":"146","author":[{"given":"Rabeah","family":"Bayazid","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Christina","family":"Orru\u2019","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rabail","family":"Aslam","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yvonne","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Amelia","family":"Silva-Rohwer","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seong-Ki","family":"Lee","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rossana","family":"Occhipinti","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Qingzhong","family":"Kong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shashirekha","family":"Shetty","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Mark L.","family":"Cohen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Byron","family":"Caughey","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lawrence B.","family":"Schonberger","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian S.","family":"Appleby","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5770-3848","authenticated-orcid":false,"given":"Ignazio","family":"Cali","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2023,5,8]]},"reference":[{"key":"2581_CR1","doi-asserted-by":"publisher","first-page":"208","DOI":"10.1001\/archneurol.2008.533","volume":"66","author":"BS Appleby","year":"2009","unstructured":"Appleby BS, Appleby KK, Crain BJ, Onyike CU, Wallin MT, Rabins PV (2009) Characteristics of established and proposed sporadic Creutzfeldt\u2013Jakob disease variants. 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Appleby has received research funding from CDC, NIH, Ionis, and Alector. He is consultant for Acadia, Ionis, Sangamo, and Merck. Received royalties from Wolters Kluwer. Drs. Orru\u2019 and Caughey have submitted patent applications relating to RT-QuIC assays. All the other authors declare that they have no competing interests.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}},{"value":"All procedures were conducted under protocols approved by the Institutional Review Board at CWRU. In all cases, written informed consent for research was obtained from the patient or legal guardian and the material used had appropriate ethical approval for use in this study. All patient data and samples were coded and handled according to NIH guidelines to protect patient identities. Animal studies were performed under the protocols approved by CWRU Institutional Animal Care and Use Committee (IACUC).","order":3,"name":"Ethics","group":{"name":"EthicsHeading","label":"Ethics approval and consent to participate"}}]}}