中文摘要：

神经退行性疾病是一类导致神经元细胞退化、功能丧失的疾病。随着RNA结合蛋白TDP-43和FUS被发现与神经退行性疾病渐冻人症（amyotrophic lateral sclerosis,ALS）密切相关,人们越来越多地关注RNA结合蛋白与神经退行性疾病的关系。大多数RNA结合蛋白都存在一个类似于prion的结构域,这个结构域使其容易发生积聚,并与神经毒性的产生相关。RNA结合蛋白参与应激颗粒的形成,应激颗粒的形成可能与神经退行性疾病相关,这进一步揭示了RNA结合蛋白在这类疾病中可能发挥作用。

英文摘要：

Neurodegenerative diseases are a class of diseases that can result in neuronal cell degeneration and loss of their functions. RNA binding proteins （RBPs）, such as TDP-43 and FUS, have been found closely associated with amyotrophic lateral sclerosis （ALS） several years ago. Since then, the relationship between RNA binding proteins and neurodegenerative diseases has been widely concerned. A prion-like domain existed in the majority of RNA binding proteins is aggregation-prone and may cause neuronal toxicity. Interestingly, some evidence indicated that RNA binding proteins participate in the formation of stress granules in cytoplasm. The appearance of the stress granules accompanied with RBPs in many neurodegenerative diseases further demonstrated the potential physiological roles of RNA binding proteins in these diseases.