The Roslin Institute and Royal (Dick) School of Veterinary Studies, Midlothian, UK.
Prion. 2012 Sep-Oct;6(4):322-33. doi: 10.4161/pri.20676. Epub 2012 Aug 16.
Prion diseases are subacute neurodegenerative diseases that affect humans and a range of domestic and free-ranging animal species. These diseases are characterized by the accumulation of PrP (Sc), an abnormally folded isoform of the cellular prion protein (PrP (C)), in affected tissues. The pathology during prion disease appears to occur almost exclusively within the central nervous system. The extensive neurodegeneration which occurs ultimately leads to the death of the host. An intriguing feature of the prion diseases, when compared with other protein-misfolding diseases, is their transmissibility. Following peripheral exposure, some prion diseases accumulate to high levels within lymphoid tissues. The replication of prions within lymphoid tissue has been shown to be important for the efficient spread of disease to the brain. This article describes recent progress in our understanding of the cellular mechanisms that influence the propagation of prions from peripheral sites of exposure (such as the lumen of the intestine) to the brain. A thorough understanding of these events will lead to the identification of important targets for therapeutic intervention, or alternatively, reveal additional processes that influence disease susceptibility to peripherally-acquired prion diseases.
朊病毒病是亚急性神经退行性疾病，影响人类和一系列家养和野生动物物种。这些疾病的特征是在受影响的组织中积累 PrP（Sc），即细胞朊病毒蛋白（PrP（C））的异常折叠同工型。朊病毒病期间的病理学似乎几乎仅在中枢神经系统中发生。广泛的神经退行性变最终导致宿主死亡。与其他蛋白质错误折叠疾病相比，朊病毒病的一个有趣特征是其传染性。在周围暴露后，一些朊病毒病在淋巴组织中积累到很高的水平。已经表明，在淋巴组织内复制朊病毒对于疾病向大脑的有效传播很重要。本文描述了我们对影响朊病毒从周围暴露部位（例如肠道腔）传播到大脑的细胞机制的最新理解进展。对这些事件的透彻了解将导致确定治疗干预的重要目标，或者揭示影响获得性朊病毒病易感性的其他过程。
