肌萎缩侧索硬化症(ALS)目前仍面临发病机制不明及无有效治疗措施的困境,研究发现ALS中存在显著的蛋白稳态失调,包括蛋白质错误折叠和异常蛋白聚集体的形成,对ALS的起病及发展具有重要作用。其中,作为分子伴侣家族的热休克蛋白(HSP)因具有维持蛋白稳态、促进异常蛋白聚集体降解的作用而受到了越来越多的关注。
At present, the pathogenesis of amyotrophic lateral sclerosis(ALS) is still unclear and there is no effective treatment. Studies have found that there are significant protein homeostasis disorders in ALS, including protein misfolding and the formation of abnormal protein aggregates, which play an important role in the occurrence and development of ALS. As a family of molecular chaperones, heat shock proteins(HSPs) have received increasing attention due to their roles in maintaining protein homeostasis and promoting the degradation of abnormal protein aggregates.