Mitochondrial diseases (encephalomyopathies) have traditionally been ascribed to defects of the respiratory chain, which has helped researchers explain their genetic and clinical complexity. However, other mitochondrial functions are greatly important for the nervous system, including protein importation, organellar dynamics, and programmed cell death. Defects in genes controlling these functions are attracting increasing attention as causes not only of neurological (and psychiatric) diseases but also of age-related neurodegenerative disorders. After discussing some pathogenic conundrums regarding the neurological manifestations of the respiratory chain defects, we review altered mitochondrial dynamics in the etiology of specific neurological diseases and in the physiopathology of more common neurodegenerative disorders.

中文翻译：

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神经系统中的线粒体疾病。

线粒体疾病（脑脊髓病）传统上归因于呼吸链的缺陷，这有助于研究人员解释其遗传和临床复杂性。但是，其他线粒体功能对神经系统也非常重要，包括蛋白质输入，细胞器动力学和程序性细胞死亡。控制这些功能的基因缺陷越来越引起人们的关注，不仅是神经系统疾病（和精神疾病）的原因，还包括与年龄有关的神经退行性疾病的原因。在讨论了有关呼吸链缺陷的神经系统表现的一些致病难题之后，我们回顾了特定神经系统疾病的病因学和更常见的神经退行性疾病的生理病理学中改变的线粒体动力学。