Autophagy as a common pathway in amyotrophic lateral sclerosis.

Autophagy as a common pathway in amyotrophic lateral sclerosis.
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DOI:
10.1016/j.neulet.2018.04.006
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发表时间:
2019-04-01
影响因子:
2.5
通讯作者:
Wang J
Wang J
中科院分区:
医学4区
文献类型:
--
作者:
Nguyen DKH;Thombre R;Wang J

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依赖性神经退行性疾病与蛋白质质量控制系统(包括自噬)的下降有关。肌萎缩侧索硬化症(ALS)是一种病因复杂的运动神经元退行性疾病,与其他神经退行性疾病如额颞叶痴呆的联系日益增加。在ALS的各种遗传原因中,一个引人注目的特征是与自噬及其相关途径的共同联系。与其他神经退行性疾病一样,蛋白质错误折叠是一个反复出现的主题,但重要的是,ALS基因之间存在一条独特的共同线索,将它们与自噬级联反应联系起来。然而,自噬在ALS中的作用仍然是个谜,目前还不清楚激活或抑制自噬是否是改善疾病的可靠途径。自噬与ALS的不同遗传形式之间的联系的主要证据进行了讨论。
Age-dependent neurodegenerative diseases are associated with a decline in protein quality control systems including autophagy. Amyotrophic lateral sclerosis (ALS) is a motor neuron degenerative disease of complex etiology with increasing connections to other neurodegenerative conditions such as frontotemporal dementia. Among the diverse genetic causes for ALS, a striking feature is the common connection to autophagy and its associated pathways. There is a recurring theme of protein misfolding as in other neurodegenerative diseases, but importantly there is a distinct common thread among ALS genes that connects them to the cascade of autophagy. However, the roles of autophagy in ALS remain enigmatic and it is still unclear whether activation or inhibition of autophagy would be a reliable avenue to ameliorate the disease. The main evidence that links autophagy to different genetic forms of ALS is discussed.
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