Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain”
In sporadic Creutzfeldt-Jakob disease, molecular subtypes are neuropathologically well identified by the lesioning profile and the immunohistochemical PrP<sup>d</sup> deposition pattern in the grey matter (histotypes). While astrocytic PrP pathology has been reported in variant CJD and s...
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| Format: | Article |
| Language: | English |
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MDPI AG
2021-09-01
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| Series: | Viruses |
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| Online Access: | https://www.mdpi.com/1999-4915/13/9/1796 |
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| author | Ellen Gelpi Sigrid Klotz Nuria Vidal-Robau Gerda Ricken Günther Regelsberger Thomas Ströbel Ognian Kalev Marlene Leoni Herbert Budka Gabor G. Kovacs |
| author_facet | Ellen Gelpi Sigrid Klotz Nuria Vidal-Robau Gerda Ricken Günther Regelsberger Thomas Ströbel Ognian Kalev Marlene Leoni Herbert Budka Gabor G. Kovacs |
| author_sort | Ellen Gelpi |
| collection | DOAJ |
| description | In sporadic Creutzfeldt-Jakob disease, molecular subtypes are neuropathologically well identified by the lesioning profile and the immunohistochemical PrP<sup>d</sup> deposition pattern in the grey matter (histotypes). While astrocytic PrP pathology has been reported in variant CJD and some less frequent histotypes (e.g., MV2K), oligodendroglial pathology has been rarely addressed. We assessed a series of sCJD cases with the aim to identify particular histotypes that could be more prone to harbor oligodendroglial PrP<sup>d</sup>. Particularly, the MM2C phenotype, in both its more “pure” and its mixed MM1+2C or MV2K+2C forms, showed more frequent oligodendroglial PrP pathology in the underlying white matter than the more common MM1/MV1 and VV2 histotypes, and was more abundant in patients with a longer disease duration. We concluded that the MM2C strain was particularly prone to accumulate PrP<sup>d</sup> in white matter oligodendrocytes. |
| first_indexed | 2024-03-10T07:08:56Z |
| format | Article |
| id | doaj.art-b4323e26672c4c9483ca3373b6d24376 |
| institution | Directory Open Access Journal |
| issn | 1999-4915 |
| language | English |
| last_indexed | 2024-03-10T07:08:56Z |
| publishDate | 2021-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Viruses |
| spelling | doaj.art-b4323e26672c4c9483ca3373b6d243762023-11-22T15:38:07ZengMDPI AGViruses1999-49152021-09-01139179610.3390/v13091796Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain”Ellen Gelpi0Sigrid Klotz1Nuria Vidal-Robau2Gerda Ricken3Günther Regelsberger4Thomas Ströbel5Ognian Kalev6Marlene Leoni7Herbert Budka8Gabor G. Kovacs9Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaDepartment of Neurology, Bellvitge University Hospital, L’Hospitalet de Llobregat, 08908 Barcelona, SpainDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaDepartment of Neuropathology/Pathology, Kepler Universitäts Klinikum, 4020 Linz, AustriaDepartment of Neuropathology/Pathology, Medical University Graz, 8036 Graz, AustriaDivision of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, AustriaTanz Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto, ON M5T 0S8, CanadaIn sporadic Creutzfeldt-Jakob disease, molecular subtypes are neuropathologically well identified by the lesioning profile and the immunohistochemical PrP<sup>d</sup> deposition pattern in the grey matter (histotypes). While astrocytic PrP pathology has been reported in variant CJD and some less frequent histotypes (e.g., MV2K), oligodendroglial pathology has been rarely addressed. We assessed a series of sCJD cases with the aim to identify particular histotypes that could be more prone to harbor oligodendroglial PrP<sup>d</sup>. Particularly, the MM2C phenotype, in both its more “pure” and its mixed MM1+2C or MV2K+2C forms, showed more frequent oligodendroglial PrP pathology in the underlying white matter than the more common MM1/MV1 and VV2 histotypes, and was more abundant in patients with a longer disease duration. We concluded that the MM2C strain was particularly prone to accumulate PrP<sup>d</sup> in white matter oligodendrocytes.https://www.mdpi.com/1999-4915/13/9/1796Creutzfeldt-Jakob diseaseCJDPrPprionhistotypeglia |
| spellingShingle | Ellen Gelpi Sigrid Klotz Nuria Vidal-Robau Gerda Ricken Günther Regelsberger Thomas Ströbel Ognian Kalev Marlene Leoni Herbert Budka Gabor G. Kovacs Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” Viruses Creutzfeldt-Jakob disease CJD PrP prion histotype glia |
| title | Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” |
| title_full | Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” |
| title_fullStr | Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” |
| title_full_unstemmed | Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” |
| title_short | Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain” |
| title_sort | histotype dependent oligodendroglial prp pathology in sporadic cjd a frequent feature of the m2c strain |
| topic | Creutzfeldt-Jakob disease CJD PrP prion histotype glia |
| url | https://www.mdpi.com/1999-4915/13/9/1796 |
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