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中文摘要：
朊病毒病是一组蛋白质折叠异常的蛋白质构象病,由正常朊蛋白PrP^C转化为具蛋白酶抗性的异常朊蛋白PrP^Sc并在中枢神经系统聚集引起.其主要的病理变化为神经元的丢失,脑组织的海绵样变及神经胶质细胞增生.目前认为其神经元丢失的主要原因为凋亡及自噬等,而神经元的凋亡涉及多种因素及途径,机制较为复杂.对神经元凋亡的研究将有助于揭示朊病毒病的发病机制.
英文摘要：
Prion diseases are a group of protein conformational diseases attributable to inaccurate protein folding. They are caused by the accumulation of the abnormal protease resistant prion protein （PrP^Sc） from the post-translational conversion of the normal host-encoded PrP^C in the central nervous system （CNS） . Prion diseases are characterized by neuronal loss, spongiosis, reactive astrocytosis, and microgliosis. At present it is thought that the main cause of neuronal loss is apoptosis and autophagy, while neuronal apoptosis is associated with many factors and pathways. The study of neuronal apoptosis contributes to the unearthing of the mechanism of prion diseases.
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Human prion protein mutants with deleted and inserted octarepeats undergo in different pathways to t
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