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首页> 外文期刊>Redox Biology >Oxidative stress, mitochondrial abnormalities and antioxidant defense in Ataxia-telangiectasia, Bloom syndrome and Nijmegen breakage syndrome
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Oxidative stress, mitochondrial abnormalities and antioxidant defense in Ataxia-telangiectasia, Bloom syndrome and Nijmegen breakage syndrome

机译：共济失调毛细血管扩张症，布鲁姆综合征和奈梅亨断裂综合征的氧化应激，线粒体异常和抗氧化防御

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Rare pleiotropic genetic disorders, Ataxia-telangiectasia (A-T), Bloom syndrome (BS) and Nijmegen breakage syndrome (NBS) are characterised by immunodeficiency, extreme radiosensitivity, higher cancer susceptibility, premature aging, neurodegeneration and insulin resistance. Some of these functional abnormalities can be explained by aberrant DNA damage response and chromosomal instability. It has been suggested that one possible common denominator of these conditions could be chronic oxidative stress caused by endogenous ROS overproduction and impairment of mitochondrial homeostasis. Recent studies indicate new, alternative sources of oxidative stress in A-T, BS and NBS cells, including NADPH oxidase 4 (NOX4), oxidised low-density lipoprotein (ox-LDL) or Poly (ADP-ribose) polymerases (PARP). Mitochondrial abnormalities such as changes in the ultrastructure and function of mitochondria, excess mROS production as well as mitochondrial damage have also been reported in A-T, BS and NBS cells. A-T, BS and NBS cells are inextricably linked to high levels of reactive oxygen species (ROS), and thereby, chronic oxidative stress may be a major phenotypic hallmark in these diseases. Due to the presence of mitochondrial disturbances, A-T, BS and NBS may be considered mitochondrial diseases. Excess activity of antioxidant enzymes and an insufficient amount of low molecular weight antioxidants indicate new pharmacological strategies for patients suffering from the aforementioned diseases. However, at the current stage of research we are unable to ascertain if antioxidants and free radical scavengers can improve the condition or prolong the survival time of A-T, BS and NBS patients. Therefore, it is necessary to conduct experimental studies in a human model.
机译：罕见的多效性遗传疾病，共济失调-毛细血管扩张症（A-T），布卢姆综合征（BS）和奈梅亨断裂综合征（NBS）的特征是免疫缺陷，极端放射敏感性，较高的癌症易感性，早衰，神经退行性变和胰岛素抵抗。这些功能异常中的一些可以通过异常的DNA损伤反应和染色体不稳定来解释。有人提出，这些情况的一个可能的共同点可能是内源性ROS过量产生和线粒体稳态失衡所引起的慢性氧化应激。最近的研究表明，A-T，BS和NBS细胞中存在新的氧化应激源，包括NADPH氧化酶4（NOX4），氧化的低密度脂蛋白（ox-LDL）或聚（ADP-核糖）聚合酶（PARP）。线粒体异常如线粒体的超微结构和功能的变化，过量的mROS产生以及线粒体损伤也已在A-T，BS和NBS细胞中报道。 A-T，BS和NBS细胞与高水平的活性氧（ROS）有着千丝万缕的联系，因此，慢性氧化应激可能是这些疾病的主要表型特征。由于线粒体紊乱的存在，A-T，BS和NBS可能被认为是线粒体疾病。抗氧化剂酶的活性过高和低分子量抗氧化剂的量不足，为患有上述疾病的患者提供了新的药理策略。但是，在目前的研究阶段，我们无法确定抗氧化剂和自由基清除剂是否可以改善A-T，BS和NBS患者的病情或延长其生存时间。因此，有必要在人体模型中进行实验研究。

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