Jagaraj Cyril Jones, Parakh Sonam, Atkin Julie D
Department of Biomedical Sciences, Macquarie University Centre for MND Research, Faculty of Medicine and Health Sciences, Macquarie University, Sydney, NSW, Australia.
Department of Biochemistry and Genetics, La Trobe Institute for Molecular Science, La Trobe University, Bundoora, VIC, Australia.
Front Cell Neurosci. 2021 Feb 18;14:581950. doi: 10.3389/fncel.2020.581950. eCollection 2020.
The cellular redox state, or balance between cellular oxidation and reduction reactions, serves as a vital antioxidant defence system that is linked to all important cellular activities. Redox regulation is therefore a fundamental cellular process for aerobic organisms. Whilst oxidative stress is well described in neurodegenerative disorders including amyotrophic lateral sclerosis (ALS), other aspects of redox dysfunction and their contributions to pathophysiology are only just emerging. ALS is a fatal neurodegenerative disease affecting motor neurons, with few useful treatments. Hence there is an urgent need to develop more effective therapeutics in the future. Here, we discuss the increasing evidence for redox dysregulation as an important and primary contributor to ALS pathogenesis, which is associated with multiple disease mechanisms. Understanding the connection between redox homeostasis, proteins that mediate redox regulation, and disease pathophysiology in ALS, may facilitate a better understanding of disease mechanisms, and lead to the design of better therapeutic strategies.
细胞氧化还原状态，即细胞氧化与还原反应之间的平衡，是一种至关重要的抗氧化防御系统，与所有重要的细胞活动相关联。因此，氧化还原调节是需氧生物的一个基本细胞过程。虽然氧化应激在包括肌萎缩侧索硬化症（ALS）在内的神经退行性疾病中已有充分描述，但氧化还原功能障碍的其他方面及其对病理生理学的贡献才刚刚显现出来。ALS是一种影响运动神经元的致命性神经退行性疾病，有效治疗方法很少。因此，未来迫切需要开发更有效的治疗方法。在这里，我们讨论了越来越多的证据表明氧化还原失调是ALS发病机制的一个重要且主要的促成因素，这与多种疾病机制相关。了解氧化还原稳态、介导氧化还原调节的蛋白质与ALS疾病病理生理学之间的联系，可能有助于更好地理解疾病机制，并促成更好治疗策略的设计。
