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Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain”
| Content Provider | MDPI |
|---|---|
| Author | Thomas, Ströbel Gelpi, Ellen Klotz, Sigrid Vidal-Robau, Nuria Ricken, Gerda Regelsberger, Günther Kalev, Ognian Leoni, Marlene Budka, Herbert Kovacs, Gabor G. |
| Copyright Year | 2021 |
| Description | In sporadic Creutzfeldt-Jakob disease, molecular subtypes are neuropathologically well identified by the lesioning profile and the immunohistochemical $PrP^{d}$ 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 $PrP^{d}$. 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 $PrP^{d}$ in white matter oligodendrocytes. |
| Starting Page | 1796 |
| e-ISSN | 19994915 |
| DOI | 10.3390/v13091796 |
| Journal | Viruses |
| Issue Number | 9 |
| Volume Number | 13 |
| Language | English |
| Publisher | MDPI |
| Publisher Date | 2021-09-09 |
| Access Restriction | Open |
| Subject Keyword | Viruses Creutzfeldt-jakob Disease Cjd Prp Prion Histotype Glia Oligodendrocytes |
| Content Type | Text |
| Resource Type | Article |