Histotype-Dependent Oligodendroglial PrP Pathology in Sporadic CJD: A Frequent Feature of the M2C “Strain”

Viruses

Nuria Vidal-Robau¹  Ognian Kalev²  Marlene Leoni³  Günther Regelsberger⁴  Herbert Budka⁴  Ellen Gelpi⁴  Thomas Ströbel⁴  Sigrid Klotz⁴  Gerda Ricken⁴  Gabor G. Kovacs⁵ 

[1] Department of Neurology, Bellvitge University Hospital, L’Hospitalet de Llobregat, 08908 Barcelona, Spain;Department of Neuropathology/Pathology, Kepler Universitäts Klinikum, 4020 Linz, Austria;Department of Neuropathology/Pathology, Medical University Graz, 8036 Graz, Austria;Division of Neuropathology and Neurochemistry, Department of Neurology, Medical University of Vienna, 1090 Vienna, Austria;Tanz Centre for Research in Neurodegenerative Disease, University of Toronto, Toronto, ON M5T 0S8, Canada;

关键词: Creutzfeldt-Jakob disease;    CJD;    PrP;    prion;    histotype;    glia;   

DOI  :  10.3390/v13091796

来源: DOAJ

【 摘 要 】

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.

【 授权许可】

Unknown