报告题目： Prions and neurodegenerative diseases

报告人： Prof. Wenquan Zou （邹文泉 教授）Departments of Pathology and Neurology, Case Western Reserve University, Cleveland, Ohio, USA

报告时间：2016年7月7日（周四）10:00

报告地点：逸夫楼C座314会议室

摘要：

Since the term protein was first coined in 1838 and discovered to be the essential component of fibrin and albumin, all cellular proteins were presumed to serve beneficial roles in plants and mammals. However, in 1967, Griffith proposed that proteins could be infectious pathogens and postulated their involvement in scrapie, a universally fatal transmissible spongiform encephalopathy in goats and sheep. Nevertheless, this novel hypothesis had not been evidenced until 1982 when Prusiner and co-workers purified infectious particles from scrapie-infected hamster brains and demonstrated that they consisted of a specific protein which he called “prion”. Unprecedentedly, the infectious protein pathogen is actually derived from its endogenous cellular form in the central nervous system. Compared with other infectious agents such as bacteria, viruses, and fungi, prions do not contain genetic materials such as DNA or RNA. The unique traits and genetic information of prions are believed to be encoded within the conformational structure and post-translational modifications of the protein. Now it is known that prions are associated with a group of fatal transmissible spongiform encephalopathy not only in animals such as scrapie and mad cow disease but also in humans including Creutzfeldt-Jakob disease, Gerstmann-Str"aussler-Scheinker Syndrome and kuru. Remarkably, prion-like pathogenetic mechanism has been recently observed in other neurodegenerative diseases such as Alzheimer’s disease, Parkinson’s disease and amyotrophic lateral sclerosis that are all associated with different transmissible proteins. In my talk, I will give a brief introduction of aging-related neurodegenerative diseases including prion diseases and will present our own work on infectious proteins and prion diseases (Supported by the National Institutes of Health NS062787, NS087588, and NS096626 as well as the CJD Foundation.)