氧化应激与亨廷顿舞蹈症:益处、危害与严峻之处

Oxidative Stress and Huntington's Disease: The Good, The Bad, and The Ugly.

作者信息

Kumar Amit, Ratan Rajiv R

机构信息

Burke Medical Research Institute, White Plains, NY, USA.

Brain and Mind Research Institute, Weill Medical College of Cornell University, New York, NY, USA.

出版信息

J Huntingtons Dis. 2016 Oct 1;5(3):217-237. doi: 10.3233/JHD-160205.

Abstract

Redox homeostasis is crucial for proper cellular functions, including receptor tyrosine kinase signaling, protein folding, and xenobiotic detoxification. Under basal conditions, there is a balance between oxidants and antioxidants. This balance facilitates the ability of oxidants, such as reactive oxygen species, to play critical regulatory functions through a direct modification of a small number of amino acids (e.g. cysteine) on signaling proteins. These signaling functions leverage tight spatial, amplitude, and temporal control of oxidant concentrations. However, when oxidants overwhelm the antioxidant capacity, they lead to a harmful condition of oxidative stress. Oxidative stress has long been held to be one of the key players in disease progression for Huntington's disease (HD). In this review, we will critically review this evidence, drawing some intermediate conclusions, and ultimately provide a framework for thinking about the role of oxidative stress in the pathophysiology of HD.

摘要

氧化还原稳态对于细胞的正常功能至关重要,包括受体酪氨酸激酶信号传导、蛋白质折叠和外源性物质解毒。在基础条件下,氧化剂和抗氧化剂之间存在平衡。这种平衡有助于氧化剂(如活性氧)通过直接修饰信号蛋白上的少数氨基酸(如半胱氨酸)来发挥关键的调节功能。这些信号功能利用了对氧化剂浓度的严格空间、幅度和时间控制。然而,当氧化剂超过抗氧化能力时,就会导致氧化应激的有害状态。长期以来,氧化应激一直被认为是亨廷顿舞蹈病(HD)疾病进展的关键因素之一。在这篇综述中,我们将批判性地审视这一证据,得出一些中间结论,并最终提供一个思考氧化应激在HD病理生理学中作用的框架。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e8b5/5310831/0becbd540ac4/nihms847717f1.jpg

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