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本文引用的文献

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Familial cardiomyopathy with cataracts and lactic acidosis: a defect in complex I (NADH-dehydrogenase) of the mitochondria respiratory chain.伴有白内障和乳酸性酸中毒的家族性心肌病:线粒体呼吸链复合体I(NADH脱氢酶)缺陷
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Apoptosis and increased generation of reactive oxygen species in Down's syndrome neurons in vitro.唐氏综合征神经元在体外的凋亡及活性氧生成增加
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Optimized steps in fluorometric determination of thiobarbituric acid-reactive substances in serum: importance of extraction pH and influence of sample preservation and storage.血清中硫代巴比妥酸反应性物质荧光测定的优化步骤:提取pH值的重要性以及样品保存和储存的影响
Clin Chem. 1993 Dec;39(12):2522-6.
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Oxidative stress, glutamate, and neurodegenerative disorders.氧化应激、谷氨酸与神经退行性疾病。
Science. 1993 Oct 29;262(5134):689-95. doi: 10.1126/science.7901908.
5
Do defects in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases?线粒体能量代谢缺陷是神经退行性疾病病理学的基础吗?
Trends Neurosci. 1993 Apr;16(4):125-31. doi: 10.1016/0166-2236(93)90117-5.
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The role of free radicals and p53 in neuron apoptosis in vivo.体内自由基和p53在神经元凋亡中的作用。
J Neurosci. 1995 Aug;15(8):5851-7. doi: 10.1523/JNEUROSCI.15-08-05851.1995.
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A radical hypothesis for neurodegeneration.一种关于神经退行性变的激进假说。
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8
Bioenergetic and oxidative stress in neurodegenerative diseases.神经退行性疾病中的生物能量与氧化应激
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9
Iron and oxygen: a biologically damaging mixture.铁与氧:一种具有生物破坏性的混合物。
Acta Paediatr Scand Suppl. 1989;361:78-85. doi: 10.1111/apa.1989.78.s361.78.

血浆丙二醛升高与小脑结构缺陷相关。

Increased plasma malondialdehyde associated with cerebellar structural defects.

作者信息

Ramaekers V T, Bosman B, Jansen G A, Wanders R J

机构信息

Department of Paediatrics, University Hospital Aachen, Germany.

出版信息

Arch Dis Child. 1997 Sep;77(3):231-4. doi: 10.1136/adc.77.3.231.

DOI:10.1136/adc.77.3.231
PMID:9370902
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1717310/
Abstract

BACKGROUND

Malondialdehyde (MDA) in plasma is regarded as an indicator for increased lipid peroxidation.

METHOD

Measurements of MDA concentrations in plasma were compared among healthy children (n = 31), patients with neurological disorders or epileptic syndromes (n = 15), and children with pontocerebellar structural defects (n = 31), where the cause or genetic defect remained unknown.

RESULTS

In healthy children the median MDA value was 5.86 nmol/ml (mean (SD) value: 6.25 (1.97), range: 3.76-11.19). For the group with various neurological disorders or epilepsy, the values were similar with the median value at 5.66 nmol/ml (range 0.22-10.86). Compared with healthy controls and the neurological/ epileptic group, the 31 children with pontocerebellar structural defects had significantly increased MDA values with a median value at 11.29 nmol/ml (mean (SD) value: 11.62 (3.27), range 3.65-19.22).

IMPLICATION

These findings of increased plasma MDA in the majority of children with pontocerebellar structural defects of unknown origin raised the question whether increased lipid peroxidation leads to prenatal and postnatal pontocerebellar maldevelopment or degeneration.

摘要

背景

血浆中的丙二醛(MDA)被视为脂质过氧化增加的指标。

方法

比较了健康儿童(n = 31)、患有神经系统疾病或癫痫综合征的患者(n = 15)以及脑桥小脑结构缺陷儿童(n = 31)的血浆MDA浓度,这些脑桥小脑结构缺陷儿童的病因或基因缺陷尚不清楚。

结果

健康儿童的MDA中位数为5.86 nmol/ml(均值(标准差):6.25(1.97),范围:3.76 - 11.19)。患有各种神经系统疾病或癫痫的组,数值与之相似,中位数为5.66 nmol/ml(范围0.22 - 10.86)。与健康对照组和神经/癫痫组相比,31例脑桥小脑结构缺陷儿童的MDA值显著升高,中位数为11.29 nmol/ml(均值(标准差):11.62(3.27),范围3.65 - 19.22)。

启示

大多数病因不明的脑桥小脑结构缺陷儿童血浆MDA升高的这些发现,引发了脂质过氧化增加是否导致产前和产后脑桥小脑发育不良或退化的问题。