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(American Journal of Pathology. 2002;161:1979-1984.)
© 2002 American Society for Investigative Pathology

Short Communications

Disease-Associated Prion Protein in Vessel Walls

Oskar Koperek^*, Gábor G. Kovács^*, Diane Ritchie, James W. Ironside, Herbert Budka^* and Georg Wick

From the Institute of Neurology,^* University of Vienna, and Austrian Reference Center for Human Prion Diseases, Vienna, Austria; the National Creutzfeldt-Jakob Disease Surveillance Unit and the Department of Pathology, University of Edinburgh, Western General Hospital, Edinburgh, Scotland, United Kingdom; and the Institute of Pathophysiology, University of Innsbruck Medical School, and the Institute for Biomedical Aging Research, Austrian Academy Science, Innsbruck, Austria

Human prion diseases like Creutzfeldt-Jakob disease are infectious, inherited, or sporadic neurodegenerative disorders, characterized by the accumulationof an abnormal isoform of the host-encoded prion protein. This affects nervous tissue in sporadic Creutzfeldt-Jakob disease and, additionally, in lymphoid tissue in bovine spongiform encephalopathy-linked variant Creutzfeldt-Jakob disease. Experimental studies have established the involvement of cells of the lymphoid and peripheral nervous system in the transport of prions to their target central nervous system tissue. To evaluate the role of vessel wall-associated mobile cells, we obtained formalin-fixed tissue blocks from various brain regions and/or basal arteries from sporadic, variant and iatrogenic Creutzfeldt-Jakob disease, and unselected control cases. We demonstrate disease-associated prion protein deposits in intracranial vessel walls, in sporadic and variant Creutzfeldt-Jakob disease by performing immunohistochemical staining and paraffin-embedded tissue blotting. Using double immunofluorescence, these deposits co-localize with HLA-DR and S-100 immunoreactive cells in the intima, which are components of the vascular-associated dendritic cell network, as well as with HLA-DR and CD-68 immunopositive macrophages of the intima and media. We conclude that mobile cells in vessel walls like dendritic and monocyte/macrophage lineage cells may be involved in spread of disease-associated prion protein and possibly also of infectivity.

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