Development and application of new diagnostic assays for the detection of prion proteins in transmissible spongiform encephalopathies
Transmissible spongiform encephalopathies (TSEs), well known as prion diseases, are fatal neurodegenerative disorders in humans and animals, which a prion protein (PrP) mainly implicates with the TSE pathogenesis. The normal cellular PrP isoform, referred to as PrPC, predominantly forms an α-helical structure. A structural alteration of the PrPC isoform can misfold into the infectious and pathogenic PrP isoform, referred to as PrPSc (or PrPD as disease-associated PrP). The PrPSc isoform consists of a β-sheet rich structure and accumulates in the central nervous system (CNS). The structural change ...
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