Milhavet Ollivier, Lehmann Sylvain
Institut de Génétique Humaine, CNRS U.P.R. 1142, 141, rue de la Cardonille, 34396 Cedex 5, Montpellier, France.
Brain Res Brain Res Rev. 2002 Feb;38(3):328-39. doi: 10.1016/s0165-0173(01)00150-3.
Transmissible spongiform encephalopathies form a group of fatal neurodegenerative disorders that have the unique property of being infectious, sporadic or genetic in origin. These diseases are believed to be the consequence of the conformational conversion of the prion protein into an abnormal isoform. Their exact pathogenic mechanism remains uncertain, but it is believed that oxidative stress plays a central role. In this article, we will first review in detail the data supporting the latter hypothesis. Subsequently, we will discuss the relationship between the prion protein and the cellular response to oxidative stress, attempting ultimately to link PrP function and neurodegeneration in these disorders.
传染性海绵状脑病是一组致命的神经退行性疾病，具有传染性、散发性或遗传性等独特特征。这些疾病被认为是朊病毒蛋白构象转化为异常异构体的结果。它们的确切致病机制尚不确定，但据信氧化应激起着核心作用。在本文中，我们将首先详细回顾支持后一种假设的数据。随后，我们将讨论朊病毒蛋白与细胞对氧化应激反应之间的关系，最终试图将这些疾病中朊蛋白的功能与神经退行性变联系起来。
