Sporadic Creutzfeldt-Jakob disease with glial PrP Res nuclear and perinuclear immunoreactivity

Proteinase K-resistant prion protein (PrP ) nuclear and perinuclear immunoreactivity in oligodendrocytes of the frontal cortex is found in one case of otherwise typical sporadic Creutzfeldt-Jakob disease (sCJD) type VV2a. The PrP nature of the inclusions is validated with several anti-PrP antibodies...

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Published in:Neuropathology 2018-10, Vol.38 (5), p.561-567
Main Authors: Fernández-Vega, Ivan, Díaz-Lucena, Daniela, Azkune Calle, Itxaso, Geijo, Maria, Juste, Ramon A, Llorens, Franc, Vicente Etxenausia, Ikerne, Santos-Juanes, Jorge, Zarranz Imirizaldu, Juan Jose, Ferrer, Isidro
Format: Article
Language:English
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Aged
Creutzfeldt-Jakob Syndrome - metabolism
Creutzfeldt-Jakob Syndrome - pathology
Frontal Lobe - metabolism
Frontal Lobe - pathology
Humans
Immunohistochemistry
Inclusion Bodies - pathology
Male
Neuroglia - metabolism
Neuroglia - pathology
Prion Proteins - metabolism
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Summary:Proteinase K-resistant prion protein (PrP ) nuclear and perinuclear immunoreactivity in oligodendrocytes of the frontal cortex is found in one case of otherwise typical sporadic Creutzfeldt-Jakob disease (sCJD) type VV2a. The PrP nature of the inclusions is validated with several anti-PrP antibodies directed to amino acids 130-160 (12F10), 109-112 (3F4), 97-102 (8G8) and the octarepeat region (amino acids 59-89: SAF32). Cellular identification and subcellular localization were evaluated with double- and triple-labeling immunofluorescence and confocal microscopy using antibodies against PrP, glial markers, and histone H3. Based on review of the literature and our own experience, this is a very odd situation that deserves further validation in other cases.
ISSN:0919-6544
1440-1789
DOI:10.1111/neup.12505