半胱氨酸代谢与神经元氧化还原稳态
Cysteine Metabolism in Neuronal Redox Homeostasis.
机构信息
The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
The Solomon H. Snyder Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
出版信息
Trends Pharmacol Sci. 2018 May;39(5):513-524. doi: 10.1016/j.tips.2018.02.007. Epub 2018 Mar 9.
Besides its essential role in protein synthesis, cysteine plays vital roles in redox homeostasis, being a component of the major antioxidant glutathione (GSH) and a potent antioxidant by itself. In addition, cysteine undergoes a variety of post-translational modifications that modulate several physiological processes. It is becoming increasingly clear that redox-modulated events play important roles not only in peripheral tissues but also in the brain where cysteine disposition is central to these pathways. Dysregulated cysteine metabolism is associated with several neurodegenerative disorders. Accordingly, restoration of cysteine balance has therapeutic benefits. This review discusses metabolic signaling pathways pertaining to cysteine disposition in the brain under normal and pathological conditions, highlighting recent findings on cysteine metabolism during aging and in neurodegenerative conditions such as Huntington's disease (HD) and molybdenum cofactor (MoCo) deficiency (MoCD) among others.
除了在蛋白质合成中的重要作用外，半胱氨酸在氧化还原平衡中也起着至关重要的作用，是主要抗氧化剂谷胱甘肽（GSH）的组成部分，本身也是一种有效的抗氧化剂。此外，半胱氨酸还经历了多种翻译后修饰，调节着多种生理过程。越来越清楚的是，氧化还原调节事件不仅在外周组织中而且在大脑中都起着重要作用，而半胱氨酸的分布是这些途径的核心。半胱氨酸代谢失调与几种神经退行性疾病有关。因此，恢复半胱氨酸平衡具有治疗益处。本综述讨论了正常和病理条件下大脑中与半胱氨酸分布相关的代谢信号通路，重点介绍了在衰老过程中以及在神经退行性疾病（如亨廷顿病（HD）和钼辅酶（MoCo）缺乏症（MoCD）等）中半胱氨酸代谢的最新发现。
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