Abstract
SCRAPIE and related animal and human disorders are neurodegenerative diseases1 characterized by the formation of a modified, partly proteinase-resistant protein (PrP) of the host2,3,which tends to aggregate as amyloid fibrils4 and accumulate in the brain of infected individuals5. There is a general consensus that the pathological form of PrP (PrPSc) is essential for the clinical appearance of the disease6, but whether it is part of the scrapie agent7 or a by-product of viral infection8,9is still controversial. Here we report that treatment of scrapie-infected hamsters with amphotericin B delays the accumulation in the brain of the proteinase-resistant portion of PrPSc by about 30 days without affecting scrapie replication. The consequence is that hamsters treated with amphotericin B developed clinical signs of disease later than infected controls. We argue that the proteinase-resistant portion of PrPSc is necessary for the development of the disease but that it is unlikely to be essential for scrapie replication.
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Xi, Y., Ingrosso, L., Ladogana, A. et al. Amphotericin B treatment dissociates in vivo replication of the scrapie agent from PrP accumulation. Nature 356, 598–601 (1992). https://doi.org/10.1038/356598a0
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DOI: https://doi.org/10.1038/356598a0
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