Brandner Sebastian
MRC Prion Unit, Department of Neurodegenerative Diseases, Institute of Neurology, London, UK.
Br Med Bull. 2003;66:131-9. doi: 10.1093/bmb/66.1.131.
Prion diseases or transmissible spongiform encephalopathies (TSEs) are fatal neurodegenerative diseases, clinically characterised by cognitive decline, paralleled by severe damage to the central nervous system. Prion diseases have attracted a broad interest because of their unique mechanisms of replication and propagation; however, the underlying pathogenic mechanisms are still highly speculative. In this review, current knowledge about the pathogenesis of prion diseases in the CNS will be highlighted and the most revealing animal models will be discussed, with future perspectives to address immediate questions about the pathogenesis.
朊病毒病或传染性海绵状脑病（TSEs）是致命的神经退行性疾病，临床特征为认知能力下降，同时伴有中枢神经系统的严重损伤。由于其独特的复制和传播机制，朊病毒病引起了广泛关注；然而，其潜在的致病机制仍极具推测性。在本综述中，将重点介绍目前关于中枢神经系统中朊病毒病发病机制的知识，并讨论最具启发性的动物模型，同时展望未来，以解决有关发病机制的紧迫问题。
