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Prion protein (PrP) related diseases are a heterogeneous group of fatal neurodegenerative conditions featuring aberrancies in the metabolism of the cellular prion protein (PrPC) that lead to the formation of neurotoxic or propagative conformers. Commonly referred to as transmissible spongiform encephalopaties or prion disorders, these diseases can be inherited, sporadic or infectiously acquired. The classical histopathological features of prion diseases consist in spongiform change, neuronal loss, astro- and microgliosis, and in some cases PrP amyloid deposits. Clinical manifestations can include dementia and ataxia, and are dictated by the affected CNS region as well as by the specific pattern of lesions. This chapter summarizes the contributions of mouse models to the current knowledge of the molecular basis of these diseases and discusses the potential use of some of them for prophylactic intervention.

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