Amyloidgenesis in prion disease

: Prion diseases, also known as transmissible spongiform encephalopathies, are defined as fatal, neurodegenerative, and transmissible disorders that affect both humans and other mammalian species. The central pathogenic mechanism in prion diseases is the conformational conversion of the normal prion protein, PrPC into the pathogenic prion protein, PrPSc. PrPSc, which is rich in β-sheet structure, is resistant to proteinase degradation and hence, accumulates within the brain. These processes closely relate to development of the pathology of prion diseases such as spongiform change, gliosis, and neuronal loss. Prion diseases are thought to be a type of localized amyloidosis of the brain because prion protein itself is amyloidogenic. We herein discuss prion diseases from the point of view of localized amyloidosis.