Abstract
Over 20 years ago the association of the transmissible agents of scrapie with the surface membranes and endoplasmic reticulum of infected cells was widely recognised (Hunter et al. 1971; Millson et al. 1971; Kimberlin et al. 1971). Membranes of scrapie-affected brain cells are less fluid than those found in healthy brain tissue (Viret et al. 1981), and show characteristic changes in structure when viewed by the electron microscope (Dubois-Dalcq et al. 1979). Indeed, intraneuronal membrane-bound vacuoles appear as disease-specific structures in the brains of scrapie-affected animals. Today, the question of what goes wrong with membrane biogenesis during scrapie infection remains, although it has become sharply focused on the gene structure and expression of a single membrane component, the PrP protein. This article reviews recent progress in our understanding of the biosynthesis and function of PrP and of its key role in the development of the transmissible degenerative encephalopathies (TDEs).
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Hope, J., Manson, J. (1991). The Scrapie Fibril Protein and Its Cellular Isoform. In: Chesebro, B.W. (eds) Transmissible Spongiform Encephalopathies:. Current Topics in Microbiology and Immunology, vol 172. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-76540-7_4
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