Sporadic Creutzfeldt–Jakob disease with glial PrPRes nuclear and perinuclear immunoreactivity |
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| Authors: | Ivan Fernández‐Vega Daniela Díaz‐Lucena Itxaso Azkune Calle Maria Geijo Ramon A Juste Franc Llorens Ikerne Vicente Etxenausia Jorge Santos‐Juanes Juan Jose Zarranz Imirizaldu Isidro Ferrer |
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| Institution: | 1. Pathology Department, Hospital Universitario Araba, Vitoria, Spain;2. Brain Bank Hospital Universitario Araba, Biobanco Vasco para la Investigación (O+eHun), Vitoria, Spain;3. Biomedical Research Institute of Bellvitge (IDIBELL), Hospitalet de Llobregat, Spain;4. Biomedical Network Research Center of Neurodegenerative Diseases (CIBERNED), Hospitalet de Llobregat, Spain;5. Neurology Department, Hospital Galdakao‐Usansolo, Galdakao, Spain;6. Department of Animal Health, NEIKER‐Tecnalia, Derio, Spain;7. Pathology Department, Hospital Universitario Central de Asturias, Oviedo, Spain;8. Neurology Department, Hospital Universitario de Cruces, Barakaldo, Spain;9. Department of Pathology and Experimental Therapeutics, University of Barcelona, Hospitalet de Llobregat, Spain;10. Service of Pathologic Anatomy, Bellvitge University Hospital, Hospitalet de Llobregat, Spain |
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| Abstract: | Proteinase K‐resistant prion protein (PrPRes) 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. |
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| Keywords: | astrocytes Creutzfeldt‐Jakob disease oligodendrocytes prion |
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