Shi Qingli, Gibson Gary E
Department of Neurology and Neuroscience, Weill Medical College of Cornell University, Burke Medical Research Institute, White Plains, NY 10605, USA.
Alzheimer Dis Assoc Disord. 2007 Oct-Dec;21(4):276-91. doi: 10.1097/WAD.0b013e31815721c3.
Alzheimer disease (AD) is defined by progressive impairments in memory and cognition and by the presence of extracellular neuritic plaques and intracellular neurofibrillary tangles. However, oxidative stress and impaired mitochondrial function always accompany AD. Mitochondria are a major site of production of free radicals [ie, reactive oxygen species (ROS)] and primary targets of ROS. ROS are cytotoxic, and evidence of ROS-induced damage to cell membranes, proteins, and DNA in AD is overwhelming. Nevertheless, therapies based on antioxidants have been disappointing. Thus, alternative strategies are necessary. ROS also act as signaling molecules including for transcription. Thus, chronic exposure to ROS in AD could activate cascades of genes. Although initially protective, prolonged activation may be damaging. Thus, therapeutic approaches based on modulation of these gene cascades may lead to effective therapies. Genes involved in several pathways including antioxidant defense, detoxification, inflammation, etc, are induced in response to oxidative stress and in AD. However, genes that are associated with energy metabolism, which is necessary for normal brain function, are mostly down-regulated. Redox-sensitive transcription factors such as activator protein-1, nuclear factor-kappaB, specificity protein-1, and hypoxia-inducible factor are important in redox-dependent gene regulation. Peroxisome proliferators-activated receptor-gamma coactivator (PGC-1alpha) is a coactivator of several transcription factors and is a potent stimulator of mitochondrial biogenesis and respiration. Down-regulated expression of PGC-1alpha has been implicated in Huntington disease and in several Huntington disease animal models. PGC-1alpha role in regulation of ROS metabolism makes it a potential candidate player between ROS, mitochondria, and neurodegenerative diseases. This review summarizes the current progress on how oxidative stress regulates the expression of genes that might contribute to AD pathophysiology and the implications of the transcriptional modifications for AD. Finally, potential therapeutic strategies based on the updated understandings of redox state-dependent gene regulation in AD are proposed to overcome the lack of efficacy of antioxidant therapies.
阿尔茨海默病(AD)的定义是记忆和认知功能进行性受损,以及细胞外神经炎性斑块和细胞内神经原纤维缠结的存在。然而,氧化应激和线粒体功能受损始终伴随AD出现。线粒体是自由基[即活性氧(ROS)]产生的主要场所,也是ROS的主要作用靶点。ROS具有细胞毒性,有大量证据表明ROS在AD中会对细胞膜、蛋白质和DNA造成损伤。然而,基于抗氧化剂的治疗方法一直令人失望。因此,需要其他策略。ROS还作为信号分子发挥作用,包括参与转录过程。因此,AD中慢性暴露于ROS可能会激活基因级联反应。尽管最初具有保护作用,但长期激活可能具有损害性。因此,基于调节这些基因级联反应的治疗方法可能会带来有效的治疗方案。包括抗氧化防御、解毒、炎症等在内的多种信号通路中的基因会在氧化应激反应以及AD中被诱导表达。然而,与正常脑功能所必需的能量代谢相关的基因大多表达下调。氧化还原敏感转录因子,如活化蛋白-1、核因子-κB、特异性蛋白-1和缺氧诱导因子,在氧化还原依赖性基因调控中起重要作用。过氧化物酶体增殖物激活受体γ共激活因子(PGC-1α)是多种转录因子的共激活因子,是线粒体生物发生和呼吸的有效刺激因子。PGC-1α表达下调与亨廷顿病以及多种亨廷顿病动物模型有关。PGC-1α在ROS代谢调节中的作用使其成为ROS、线粒体和神经退行性疾病之间潜在的关键因素。本综述总结了氧化应激如何调节可能导致AD病理生理学的基因表达的当前进展,以及转录修饰对AD的影响。最后,基于对AD中氧化还原状态依赖性基因调控的最新认识,提出了潜在的治疗策略,以克服抗氧化治疗缺乏疗效的问题。