血管生成素在淀粉样沉积症中发挥作用吗?（英文）

Does Angiogenin Play a Role in Amyloid Diseases?

淀粉样沉积症是致命性的疾病,可以是神经退行性的,也可以是系统性的.该疾病以错误折叠蛋白质的堆积、缠绕成纤维为特征,最终导致受累组织、器官的渐进性坏死.目前,没有有效的治疗手段可以阻止该类疾病的进程.错误折叠蛋白质的累积诱导内质网应激,被认为是退行性疾病的标志.血管生成素不仅可以调节细胞生长和增殖,也在应激条件下细胞存活中发挥作用.最近,发现血管生成素介导的应激反应可以减轻蛋白聚积造成的损伤,提示该蛋白可能在退行性疾病中具有新功能.本综述概述了血管生成素在淀粉样沉积症中的研究进展,特别是描述了血管生成素失调与该类疾病的起始和进展间的关系.我们认为,深入了解血管生成素失调的分子基础有助于发展与蛋白质错误折叠和聚积相关的退行性疾病的治疗方法.

Amyloidoses are the devastating diseases, either neurodegenerative or systemic （targeted to peripheral organs）, characterized by accumulation and aggregation of misfolded proteins into fibrils, finally leading to a progressive failure of affected tissues and organs. To date, no therapeutic approaches are available to counteract the progression of this class of diseases. Accumulation of misfolded protein aggregates increases endoplasmic reticulum stress, which can be considered a hallmark of degenerative diseases. Angiogenin, which is known to regulate cell growth and proliferation, plays a key role in sustaining cell survival under stress conditions. Recently, a new role for angiogenin in degenerative diseases is emerged, since stress response mediated by angiogenin is believed to alleviate damages inflicted by protein aggregates. This review reports some of the issues providing support to the role of angiogenin in amyloidoses and we described the recent advances toward the elucidation of the correlation between angiogenin deregulation and the onset/progression of the pathology. A detailed understanding of the molecular basis of angiogenin deregulation might lead to the development of therapies for degenerative disorders that are associated with protein misfolding and aggregation.