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The Role of Intracellular Trafficking of PrP in Prion Diseases
Xiao-Fan Wang, Jun Han, Jian-Mei Gao, Yan-Zhen Wan, Feng Li, Xiao-Ping Dong*
Prion Laboratory, National Institute for Viral Disease Control and Prevention, Chinese Center for Disease Control and Prevention, Beijing 100052, China
Abstract: Prion diseases or transmissible spongiform encephalopathies (TSEs) are a group of fatal neurodegenerative disorders that can appear in sporadic， heritable and transmissible forms. The fundamental pathogenic mechanism of prion diseases involves the post-translational conversion of PrPC into its infectious isoform PrPSc， but it remains still unclear that how prion propagation leads to apoptosis of neuronal cells. Recently more evidences show that the intracellular trafficking of PrPC may play roles in the pathogenesis of TSE. The conversion processes of PrPC to PrPSc in acquired or heritable prion diseases affect biosynthesis， intracellular localization and transport of cellular PrP， leading to neurodegeneration through perturbing normal function of PrPC or generating neurotoxic species. Possible relationship between the intracellular trafficking of PrP and molecular pathologic mechanism of prion diseases were discussed in this review.
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