146 Understanding the pathogenesis of prion and surveillance for human prion diseases in China

Through human prion disease cases, infectious animal models, cell models and prion proteins, the neuropathological and pathogenic characteristics of prion diseases, the cytotoxicity of pathological prion, the conversion and replication of prion and the infectivity of prion strains were comprehensively evaluated. With the established specimen bank of human and experimental prion diseases, several potential methodologies for diagnosis have been set up. Recently, high sensitive tools, e.g. PMCA and RT-QuIC, have been established. Since 2006, a national CJD surveillance has been conducted, covering 12 provinces in mainland China. The epidemiological, clinical and laboratory features of Chinese CJD were firstly described. Dozens of Chinese genetic prion diseases have been firstly identified.