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- W94090931 abstract "Prion diseases are neurodegenerative transmissible diseases. The infectious agent, termed prion, is thought to consist of an altered host-encoded protein. The pathogenesis of these diseases which typically in a very short time lead to rampant nerve cell death and astrocytic gliosis is poorly understood. Investigations using the in situ endlabeling technique and electron microscopy in a scrapie model in the mouse (79A strain) show that nerve cell death is due to apoptosis. A cell culture model using a synthetic peptide of the prion protein (PrP106–126) shows that this peptide is toxic only to normal neurons whereas nerve cells derived from PrP knock-out (PrP0/0) mice are unaffected by this neurotoxic effect. In addition, microglia play a crucial part in this process by secreting reactive oxygen species. Experiments in animals will have to show whether these cell culture findings adequately reflect the in vivo pathogenesis." @default.
- W94090931 created "2016-06-24" @default.
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- W94090931 date "1997-01-01" @default.
- W94090931 modified "2023-10-16" @default.
- W94090931 title "Cell death in prion disease" @default.
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- W94090931 doi "https://doi.org/10.1007/978-3-7091-6842-4_19" @default.
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