Amyotrophic lateral sclerosis (ALS) is a rare neurodegenerative disease which damages upper and lower motor neurons (UMN and LMN) innervating the muscles of the trunk, extremities, head, neck and face in cerebrum, brain stem and spinal cord, which results in the progressive weakness, atrophy and fasciculation of muscle innervated by the related UMN and LMN, accompanying with the pathological signs leaded by the cortical spinal lateral tract lesion. The pathogenesis about ALS is not fully understood, and no specific drugs are available to cure and prevent the progression of this disease at present. In this review, we reviewed the structure and associated functions of copper-zinc superoxide dismutase 1 (SOD1), discuss why SOD1 is crucial to the pathogenesis of ALS, and outline the pathogenic mechanisms of SOD1 in ALS that have been identified at recent years, including glutamate-related excitotoxicity, mitochondrial dysfunction, endoplasmic reticulum stress, oxidative stress, axonal transport disruption, prion-like propagation, and the non-cytologic toxicity of glial cells. This review will help us to deeply understand the current progression in this field of SOD1 pathogenic mechanisms in ALS.

中文翻译：

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铜锌超氧化物歧化酶 1 (SOD1) 在肌萎缩侧索硬化症中的潜在致病机制

肌萎缩侧索硬化症（ALS）是一种罕见的神经退行性疾病，它会损害支配大脑、脑干和脊髓的躯干、四肢、头、颈和面部肌肉的上运动神经元和下运动神经元（UMN 和 LMN），从而导致相关UMN和LMN所支配的肌肉进行性无力、萎缩和肌束颤动，并伴有以皮质脊髓侧束病变为主导的病理体征。ALS的发病机制尚不完全清楚，目前也没有特效药物可以治愈和预防该疾病的进展。在这篇综述中，我们回顾了铜锌超氧化物歧化酶1（SOD1）的结构和相关功能，讨论了为什么SOD1对于ALS的发病机制至关重要，并概述了近年来已发现的SOD1在ALS中的致病机制，包括谷氨酸相关的兴奋性毒性、线粒体功能障碍、内质网应激、氧化应激、轴突运输破坏、朊病毒样传播和神经胶质细胞的非细胞学毒性。本综述将有助于我们深入了解SOD1在ALS致病机制这一领域的最新进展。