中文摘要：

Prion 疾病，由海绵状的退化和错误褶层的累积描绘了并且在中央神经系统聚集了 PrPSc，是致命的 neurodegenerative 和人和动物的传染混乱之一。在更早的研究，在神经原的 autophagy 液泡经常在象 Alzheimer 的家那样的 neurodegenerative 疾病被观察， Parkinson 是，并且亨廷顿是象 prion 疾病一样的疾病。Autophagy 是几个细胞质的部件(蛋白质或细胞器) 被在一个 double-membrane-bound 泡扣押的一个高度保存的 homeostatic 过程称为的 autophagosome 并且与 lysosome 在他们的熔化之上降级了。在基础生理的层次的细胞间的自我消化的小径为维持纸巾和机关的健康地位是不可缺少的。在 prion 感染的情况下，增加证据显示 autophagy 把消除病理学的 PrPSc 的一个关键能力在神经原以内积累了。相反，在影响神经原的 autophagy 机能障碍可以贡献海绵状的变化的形成。在这评论，我们在 neurodegenerative 混乱关于哺乳动物的 autophagy 的效果总结了最近的调查结果，特别地在 prion 疾病。我们也总结了一些小分子的治疗学的潜力(例如锂， rapamycin， Sirtuin 1 和 resveratrol ) 在大脑上减轻如此的疾病的目标工作。而且，我们讨论了 autophagy 的争论角色，它是否调停 neuronal 毒性或在 neurodegenerative 混乱服务保护的功能。

英文摘要：

Prion diseases, characterized by spongiform degeneration and the accumulation of misfolded and aggregated PrPsc in the central nervous system, are one of fatal neurodegen- erative and infectious disorders of humans and animals. In earlier studies, autophagy vacuoles in neurons were frequently observed in neurodegenerative diseases such as Alzheimer＇s, Parkinson＇s, and Huntington＇s diseases as well as prion diseases. Autophagy is a highly conserved homeostatic process by which several cytoplasmic components （proteins or organelles） are sequestered in a double- membrane-bound vesicle termed ＇autophagosome＇ and degraded upon their fusion with lysosome. The pathway of intercellular self-digestion at basal physiological levels is indispensable for maintaining the healthy status of tissues and organs. In case of prion infection, increasing evidence indicates that autophagy has a crucial ability of eliminating pathological PrPsc accumulated within neurons. In contrast, autophagy dysfunction in affected neurons may contribute to the formation of spongiform changes. In this review, we summarized recent findings about the effect of mammalian autophagy in neurodegenerative disorders, particularly in prion diseases. We also summarized the therapeutic potential of some small mole- cules （such as lithium, rapamycin, Sirtuin 1 and resveratrol） targets to mitigate such diseases on brain function. Furthermore, we discussed the controversial role of autop- hagy, whether it mediates neuronal toxicity or serves a protective function in neurodegenerative disorders.