OXIDATIVE STRESS IN DEVELOPMENTAL BRAIN DISORDERS
OXIDATIVE STRESS IN DEVELOPMENTAL BRAIN DISORDERS
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DOI：
10.1007/978-1-4614-0653-2_21
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发表时间：
2012-01-01
影响因子：
3
通讯作者：
Tanuma, Naoyuki
中科院分区：
文献类型：
--
作者：
Hayashi, Masaharu;Miyata, Rie;Tanuma, Naoyuki
为了研究氧化应激在发育性脑疾病中的参与，我们在尸检脑中进行了免疫组织化学，并在患者的脑脊液和尿液中进行了酶联免疫吸附测定（ELISA）。在这里，我们回顾我们的数据遗传性DNA修复障碍，先天性代谢错误和儿童期发病的神经退行性疾病。首先，在我们对遗传性DNA修复障碍的研究中，分别在着色性干皮病、Cockayne综合征和共济失调-毛细血管扩张样疾病患者的基底节变性、脑内钙化和小脑变性中进行了DNA氧化损伤和脂质过氧化作用的增加。其次，先天性代谢异常、脂质过氧化导致的细胞凋亡似乎会导致神经元蜡样脂褐质沉积症中的神经元损伤。DNA的氧化应激和抗氧化酶的表达减少发生在大脑皮质的病变中的粘多糖沉积症和线粒体]肌病、脑病、乳酸酸中毒和中风样发作。在儿童期发病的神经退行性疾病中，增加的氧化DNA损伤和脂质过氧化可能导致脊髓性肌萎缩症中的运动神经元死亡，如肌萎缩性侧索硬化症。齿状核红核苍白球路易氏体萎缩、三联重复疾病、核苷氧化产物沉积和抗氧化酶表达减少的住院患者在豆状核中被发现。相反，氧化应激的参与在Lafora病患者中是不确定的。Rett综合征患者尿中氧化应激标志物和抗氧化能力发生变化，尽管这种变化可能与全身并发症有关。
In order to examine the involvement of oxidative stress in developmental brain disorders, we have performed immunohistochemistry in autopsy brains and enzyme-linked immunosorbent assay (ELISA) in the cerebrospinal-fluid and urines of patients. Here, we review our data on the hereditary DNA repair disorders, congenital metabolic errors and childhood-onset neurodegenerative disorders. First, in our studies on hereditary DNA repair disorders, increased oxidative DNA damage and lipid peroxidation were carried out in the degeneration of basal ganglia, intracerebral calcification and cerebellar degeneration in patients with xeroderma pigmentosum, Cockayne syndrome and ataxia-telangiectasia-like disorder, respectively. Next, congenital metabolic errors, apoptosis due to lipid peroxidation seemed to cause neuronal damage in neuronal ceroid-lipofuscinosis. Oxidative stress of DNA combined with reduced expression of antioxidant enzymes occurred in the lesion of the cerebral cortex in mucopolysaccharidoses and mitochondria] myopathy, encephalopathy, lactic acidosis and stroke-like episodes. In childhood-onset neurodegenerative disorders, increased oxidative DNA damage and lipid peroxidation may lead to motor neuron death in spinal muscular atrophy like in amyotrophic lateral sclerosis. Inpatients with dentatorubral-pallidoluysian atrophy, a triplet repeat disease, deposition of oxidative products of nucleosides and reduced expression of antioxidant enzymes were found in the lenticular nucleus. In contrast, the involvement of oxidative stress is not definite in patients with Lafora disease. Rett syndrome patients showed changes of oxidative stress markers and antioxidant power in urines, although the changes may be related to systemic complications.