PMID: 9160894Apr 1, 1997Paper

In vivo toxicity of prion protein in murine scrapie: ultrastructural and immunogold studies

Neuropathology and Applied Neurobiology
M JeffreyJ R Fraser

Abstract

Prion protein (PrP) is a cell surface, host coded, sialoglycoprotein which accumulates in excess in scrapie, Creutzfeldt-Jakob disease, bovine spongiform encephalopathy and other transmissible spongiform encephalopathies. Infection of mice with the 87 V or ME7 scrapie strains results in distinctive and very different light microscopical patterns of vacuolation and disease specific PrP accumulation. In both of these scrapie strains immunogold electron microscopy was used to locate PrP to the plasmalemma of neurons from where it was released into the neuropil. Initial PrP accumulation around neurons and in early plaques lacking amyloid fibrils was generally not associated with morphological changes either of the neuron or dendrite releasing the PrP or in the adjacent neuropil in which excess PrP accumulated. However, accumulation of pre-amyloid PrP in some brain areas was associated with specific degeneration of dendritic spines and axon terminals. Initial PrP aggregation into fibrils was also associated with tissue damage with both ME7 and 87 V plaques and diffuse accumulations. Tissue damage associated with fibrillogenesis was localized and would not be expected to have clinical significance. We conclude that pre-amyloid PrP re...Continue Reading

Citations

Aug 12, 2003·Cell Calcium·Mark Arundine, Michael Tymianski
Aug 8, 2001·The European Journal of Neuroscience·J LainéH Axelrad
Jun 7, 2000·Neuropathology and Applied Neurobiology·P V BelichenkoJ R Fraser
Feb 10, 2012·PLoS Pathogens·Cyrus BettChristina J Sigurdson
Sep 12, 2000·Current Opinion in Genetics & Development·J Hope
Apr 9, 1999·Neuropathology and Applied Neurobiology·S Betmouni, V H Perry
May 21, 2008·Neurological Research·Hiroko Ikeshima-Kataoka, Shigeki Yuasa
Oct 29, 2004·The American Journal of Pathology·Milene Russelakis-CarneiroClaudio Soto
Sep 26, 2009·The American Journal of Pathology·Zuzana SiskováVictor Hugh Perry
Nov 13, 2008·Mathematical Biosciences·Vincent CalvezBenoît Perthame
Oct 15, 2003·Neuron·Lawrence S B Goldstein
Nov 6, 2004·The Journal of Biological Chemistry·Malin K Sandberg, Peter Löw

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