摘要
Transmissible Spongiform Encephalopathies （TSE）, commonly referred to as prion diseases, are a group of rare, infectious and fatal neurodegenerative diseases in mammals. All prion diseases are thought to share a common pathogenic mechanism, which is based on the conversion of the normal cellular prion, PrPc, into the infectious and pathogenic scrapie prion protein, PrP^Sc. The accumulation of PrPsc in the CNS is then thought to impair function, induce structural damage, and cause disease. In addition to gain of toxic function, loss of normal PrPc function, a consequence of conversion to PrPsc may also contribute to pathogenesis.
