Institute of Neurological Sciences, National Research Council, Contrada Burga, Mangone, Cosenza, Italy.
Expert Rev Proteomics. 2010 Dec;7(6):907-17. doi: 10.1586/epr.10.80.
Creutzfeldt-Jakob disease (CJD) is a rare fatal neurodegenerative disease belonging to the group of transmissible spongiform encephalopathies or prion diseases. The agent responsible for the disease is the prion protein in an altered conformational form. Although there have been countless studies performed on the prion protein, the mechanisms that induce the structural change of the normal protein, and the harmful action the altered protein has on nervous cells, are still not fully understood. Furthermore, the final diagnosis for CJD can only occur with a postmortem histopathological analysis of the brain; the antemortem diagnosis is only possible for some specific CJD forms. Finally, there is no current treatment able to stop or delay the progression of the disease. Studies directed at resolving these issues are, therefore, extremely relevant. The proteomic approach is a very good strategy to be applied in such contexts because it allows easy identification of proteins and peptides possibly involved in the disease processes. In this article, the existing data regarding prion infection, biomarkers for CJD diagnosis and the use of several modern proteomic technologies for the identification of new cerebrospinal fluid polypeptides involved in CJD are reviewed.
克雅氏病（CJD）是一种罕见的致命性神经退行性疾病，属于传染性海绵状脑病或朊病毒病的范畴。导致该疾病的病原体是发生构象改变的朊蛋白。尽管已经对朊蛋白进行了无数的研究，但诱导正常蛋白结构发生变化的机制，以及异常蛋白对神经细胞的有害作用，仍未完全了解。此外，CJD 的最终诊断只能通过对大脑进行死后组织病理学分析来确定；对于某些特定的 CJD 类型，只能进行生前诊断。目前尚无能够阻止或延缓疾病进展的治疗方法。因此，解决这些问题的研究非常重要。蛋白质组学方法是解决此类问题的一种非常好的策略，因为它可以方便地鉴定可能参与疾病过程的蛋白质和肽。本文综述了朊病毒感染、CJD 诊断标志物以及几种现代蛋白质组学技术在鉴定新的脑脊液多肽中的应用，这些多肽与 CJD 有关。
