These data suggest that a major toxic effect of PrP d is at the level of plasma membranes. PrP d accumulation and internalisation from membranes is similarly associated with perturbations of membrane structure and trafficking in CNS neurons and tingible body macrophages of the LRS. Internalisation of PrP d from the chromaffin cell plasma membrane occurred in association with granule recycling following hormone exocytosis. PrP d accumulation was associated with distinctive membrane changes of chromaffin cells including increased electron density, abnormal linearity and invaginations. Using immunogold electron microscopy, non-fibrillar forms of PrP d were shown to accumulate mainly in association with chromaffin cells, occasional nerve endings and macrophages. Here we investigate the effect of sheep scrapie on the morphology and the accumulation of PrP d in the adrenal medulla of scrapie affected sheep using light and electron microscopy. Accumulations have additionally been recorded in other tissues including the peripheral nervous system and adrenal gland.
Transmissible spongiform encephalopathies (TSEs) or prion diseases are associated with accumulations of disease specific PrP (PrP d) in the central nervous system (CNS) and often the lymphoreticular system (LRS).
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