Transmissible spongiform encephalopathies, also known as prion-related diseases, are a group of fatal neurodegenerative disorders associated with the misfolding of the prion protein. In this article, the potential use of the principles of prion replication to develop novel therapeutic and diagnostic strategies for these devastating diseases are described.

Keywords:
CJD, Creutzfeldt-Jakob disease, TSE, transmissible spongiform encephalopathy, PMCA, protein misfolding cyclic amplification, bovine spongiform encephalopathy, Creutzfeldt-Jakob disease, protein misfolding, scrapie
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© 2002 Biochemical Society
2002
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