NR AFZQ
AU Jeffrey,M.J.; Goodsir,C.M.; Bruce,M.E.; McBride,P.A.; Scott,J.R.; Halliday,W.G.
TI Infection specific prion protein (PrP) accumulates on neuronal plasmalemma in scrapie infected mice
QU Neuroscience Letters 1992 Nov 23; 147(1): 106-9
PT journal article
AB Prion protein (PrP) is an abundant membrane-associated host protein which accumulates in abnormal, relatively protease-resistant forms in the brains of animals with scrapie and related diseases. Using correlative light and electron microscopy we determined the sites of subcellular localisation of PrP in mice infected with the 87V strain of scrapie. Disease specific accumulation of PrP was observed at light microscopy as amyloid plaques or as diffuse or granular staining within the neuropil, often clearly associated with individual neurons. Serial electron microscopical preparations were immunostained for PrP by the immunogold method. Gold particles were located on amyloid fibrils and on the plasmalemma of neurites at the periphery of plaques and in the neuropil, irrespective of the morphological form of PrP accumulation when viewed by light microscopy. This suggests that amyloid fibrils are formed following the accumulation and aggregation of sub-unit proteins at the plasmalemma and, furthermore, that normal PrP may be converted to its pathological form at this site.
MH Amyloid/metabolism; Amyloidosis/pathology; Animal; Cell Membrane/metabolism; Hypothalamic Area, Lateral/anatomy & histology/metabolism; Immunohistochemistry; Mice; Microscopy, Electron; Neurons/*metabolism; Prions/*biosynthesis; Scrapie/*metabolism/pathology
AD Martin J. Jeffrey (m.jeffrey@vla.maff.gov.uk), Caroline M. Goodsir, W. G. Halliday, VLA Lasswade Veterinary Laboratory, Pentlands Science Park, Bush Loan, Penicuik, Edinburgh EH26 OPZ, Scotland, UK; Moira E. Bruce (moira.bruce@bbsrc.ac.uk), Patricia A. McBride (tricia.mcbride@bbsrc.ac.uk), J. R. Scott, Institute for Animal Health, BBSRC and MRC, Neuropathogenesis Unit, Ogston Building, West Mains Road, Edinburgh EH9 3JF, Scotland, UK
SP englisch
PO Niederlande