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Extensive respiratory chain defects in inhibitory interneurones in patients with mitochondrial disease

机译:线粒体疾病患者抑制性中间神经元中广泛的呼吸链缺陷

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AimsMitochondrial disorders are among the most frequently inherited cause of neurological disease and arise due to mutations in mitochondrial or nuclear DNA. Currently, we do not understand the specific involvement of certain brain regions or selective neuronal vulnerability in mitochondrial disease. Recent studies suggest -aminobutyric acid (GABA)-ergic interneurones are particularly susceptible to respiratory chain dysfunction. In this neuropathological study, we assess the impact of mitochondrial DNA defects on inhibitory interneurones in patients with mitochondrial disease.
机译:目的线粒体疾病是神经病中最常见的遗传原因之一,是由线粒体或核DNA突变引起的。目前,我们还不了解线粒体疾病中某些大脑区域的特定参与或选择性神经元的脆弱性。最近的研究表明,-氨基丁酸(GABA)-能产生的中间神经元对呼吸链功能异常特别敏感。在这项神经病理学研究中,我们评估了线粒体DNA缺陷对线粒体疾病患者抑制性中间神经元的影响。

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