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Mechanisms of Neuroprotection by Protein Disulphide Isomerase in Amyotrophic Lateral Sclerosis

机译:蛋白二硫键异构酶在肌萎缩性侧索硬化中的神经保护机制

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Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterised by the progressive loss of motor neurons, leading to paralysis and death within several years of onset. Although protein misfolding is a key feature of ALS, the upstream triggers of disease remain elusive. Recently, endoplasmic reticulum (ER) stress was identified as an early and central feature in ALS disease models as well as in human patient tissues, indicating that ER stress could be an important process in disease pathogenesis. One important chaperone induced by ER stress is protein disulphide isomerase (PDI), which is both upregulated and posttranslationally inhibited by S-nitrosylation in ALS. In this paper, we present evidence from studies of genetics, model organisms, and patient tissues which indicate an active role for PDI and ER stress in ALS disease processes.
机译:肌萎缩性侧索硬化症(ALS)是一种毁灭性的神经退行性疾病,其特征在于运动神经元的逐渐丧失,在发病数年内导致瘫痪和死亡。尽管蛋白质错误折叠是ALS的关键特征,但疾病的上游诱因仍然难以捉摸。最近,内质网(ER)应激被认为是ALS疾病模型以及人类患者组织中的早期和中心特征,这表明ER应激可能是疾病发病机理中的重要过程。内质网应激诱导的一种重要伴侣蛋白是二硫键异构酶(PDI),它在ALS中被S-亚硝基化同时上调和翻译后抑制。在本文中,我们提供了来自遗传学,模型生物和患者组织研究的证据,这些证据表明PDI和ER应激在ALS疾病过程中发挥了积极作用。

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