Hur Kwon, Kim Jae-Il, Choi Seung-Il, Choi Eun-Kyoung, Carp Richard I, Kim Yong-Sun
Ilsong Institute of Life Science, Hallym Academy of Sciences, Hallym University, Ilsong Building, Kwanyang-dong 1605-4, Dongan-gu, Anyang 431-060, South Korea.
Mech Ageing Dev. 2002 Nov;123(12):1637-47. doi: 10.1016/s0047-6374(02)00099-4.
Transmissible spongiform encephalopathies (TSEs) or prion diseases are a group of fatal neurodegenerative diseases of humans and animals, including bovine spongiform encephalopathy (BSE) of cattle, scrapie of sheep, and Creutzfeldt-Jakob disease (CJD) of humans. Prion diseases have become an important issue in public health and in the scientific world not only due to the possible relationship between BSE and new variant CJD (nvCJD) but also due to the unique biological features of the infectious agent. Although the nature of the infectious agent and the pathogenic mechanisms of prion diseases are not fully understood, considerable evidence suggests that an abnormal form (PrP(Sc)) of a host prion protein (PrP(C)) may compose substantial parts of the infectious agent and that various factors such as oxidative stress and calcium cytotoxicity are associated with the pathogenesis of prion diseases. Here, we briefly review and discuss the pathogenic mechanisms of prion diseases. These advances in understandings of fundamental biology of prion diseases may open the possibilities for the prevention and treatment of these unusual diseases and also suggest applications in more common neurodegenerative disorders such as Alzheimer's disease (AD) and Parkinson's disease (PD).
传染性海绵状脑病（TSEs）或朊病毒病是一类人和动物的致命性神经退行性疾病，包括牛的牛海绵状脑病（BSE）、羊的痒病以及人的克雅氏病（CJD）。朊病毒病已成为公共卫生和科学界的一个重要问题，这不仅是因为BSE与新型变异型CJD（nvCJD）之间可能存在的关联，还因为感染因子独特的生物学特性。尽管感染因子的本质和朊病毒病的致病机制尚未完全明确，但大量证据表明，宿主朊病毒蛋白（PrP（C））的异常形式（PrP（Sc））可能构成感染因子的主要部分，并且诸如氧化应激和钙细胞毒性等多种因素与朊病毒病的发病机制相关。在此，我们简要回顾并讨论朊病毒病的致病机制。对朊病毒病基础生物学认识的这些进展可能为预防和治疗这些罕见疾病带来可能性，也为诸如阿尔茨海默病（AD）和帕金森病（PD）等更常见的神经退行性疾病的研究提供思路。
