Abstract:

Prions are infectious proteins formed by normal prion proteins in vivo， and prion diseases are a class of fatal neurodegenerative diseases triggered by prions. At present， although there is no clinical method to treat prion disease， a large number of researchers have studied from many angles and made some progress. This article provided a brief illustration of recent prion disease treatment options related to traditional chemical drugs， gene therapy， immunotherapy and homologous prion proteins， with an emphasis on novel drugs that target intracellular signaling pathways as well as intracellular signaling pathways of mitochondria associated with prion proteins with potential utility. This paper aimed to provide a theoretical basis for the new research direction of prion， in order to promote the clinical application of prion treatment methods.

Key words: prion disease, prion proteins, signaling pathway, gene therapy, immunotherapy

摘要：

朊病毒是一种由体内正常朊蛋白转化形成的传染性蛋白质，朊病毒病是由朊病毒引发的致命性神经退行性疾病。目前临床虽然尚无治疗朊病毒病的方法，但是大量的研究者已从多个角度进行研究，并取得了一定进展。对近期有关传统化学药物、基因治疗方法、免疫学治疗方法和同源朊蛋白的朊病毒病治疗方法进行了综述，并重点分析了新型靶向细胞内信号通路药物以及有潜在利用价值的线粒体相关朊病毒胞内作用信号通路，旨在为朊病毒新的研究方向提供理论依据，从而促进朊病毒病治疗方法应用于临床。

关键词: 朊病毒病, 朊蛋白, 基因治疗, 免疫治疗