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The pathogenesis of prion diseases is related to conformational transformation of cellular prion protein (PrP C ) into a toxic, infectious, and self-replicating conformer termed PrP Sc . Following extracerebral inoculation, the replication of PrP Sc is confined for months to years to the lymporeticular system (LRS) before the secondary CNS involvement results in occurrence...
Prion disease is characterized by a conformational change of the normal form of the prion protein (PrP C ) to the scrapie-associated form (PrP Sc ). Since the emergence of new variant Creutzfeldt-Jakob disease a potentially large human population is at risk for developing prion disease. Currently, no effective treatment or form of post-exposure prophylaxis is available for prion...
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