Mitochondria play essential metabolic roles in neural cells. Mitochondrial dysfunction has profound effects on the brain. In primary mitochondrial diseases, mutations that impair specific oxidative phosphorylation (OXPHOS) proteins or OXPHOS assembly factors lead to isolated biochemical defects and a heterogeneous group of clinical phenotypes, including mitochondrial encephalopathies. A broader defect of OXPHOS function, due to mutations in proteins involved in mitochondrial DNA maintenance, mitochondrial biogenesis, or mitochondrial tRNAs can also underlie severe mitochondrial encephalopathies. While primary mitochondrial dysfunction causes rare genetic forms of neurological disorders, secondary mitochondrial dysfunction is involved in the pathophysiology of some of the most common neurodegenerative disorders, including Alzheimer's disease, Parkinson's disease, Huntington's disease, and amyotrophic lateral sclerosis. Many studies have investigated mitochondrial function and dysfunction in bulk central nervous system (CNS) tissue. However, the interpretation of these studies has been often complicated by the extreme cellular heterogeneity of the CNS, which includes many different types of neurons and glial cells. Because neurons are especially dependent on OXPHOS for ATP generation, mitochondrial dysfunction is thought to be directly involved in cell autonomous neuronal demise. Despite being metabolically more flexible than neurons, glial mitochondria also play an essential role in the function of the CNS, and have adapted specific metabolic and mitochondrial features to support their diversity of functions. This review analyzes our current understanding and the gaps in knowledge of mitochondrial properties of glia and how they affect neuronal functions, in health and disease.

中文翻译：

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胶质线粒体功能和功能异常在健康和神经退行性疾病中。

线粒体在神经细胞中起重要的代谢作用。线粒体功能障碍对大脑有深远的影响。在原发性线粒体疾病中，损害特定氧化磷酸化（OXPHOS）蛋白或OXPHOS装配因子的突变会导致孤立的生化缺陷和一组异质的临床表型，包括线粒体脑病。由于参与线粒体DNA维持，线粒体生物发生或线粒体tRNA的蛋白质突变引起的OXPHOS功能的广泛缺陷，也可能是严重的线粒体脑病的基础。原发性线粒体功能障碍会导致罕见的遗传性神经系统疾病，而继发性线粒体功能障碍则与某些最常见的神经退行性疾病（包括阿尔茨海默氏病）的病理生理有关，帕金森氏病，亨廷顿氏病和肌萎缩性侧索硬化症。许多研究已经调查了中枢神经系统（CNS）组织中的线粒体功能和功能障碍。但是，CNS的极端细胞异质性常常使这些研究的解释复杂化，其中包括许多不同类型的神经元和神经胶质细胞。因为神经元特别依赖于OXPHOS生成ATP，所以线粒体功能障碍被认为直接参与了细胞自主神经元的死亡。尽管神经胶质线粒体在代谢上比神经元更灵活，但它在中枢神经系统的功能中也起着至关重要的作用，并适应了特定的代谢和线粒体特征以支持其功能的多样性。