Mitochondrial homeostasis is essential for maintaining healthy cellular function and survival. The detrimental involvement of mitochondrial dysfunction in neuro-degenerative diseases has recently been highlighted in human conditions, such as Parkinson's, Alzheimer's and Huntington's disease. Friedreich's ataxia (FA) is another neuro-degenerative, but also cardio-degenerative condition, where mitochondrial dysfunction plays a crucial role in disease progression. Deficient expression of the mitochondrial protein, frataxin, is the primary cause of FA, which leads to adverse alterations in whole cell and mitochondrial iron metabolism. Dys-regulation of iron metabolism in these compartments, results in the accumulation of inorganic iron deposits in the mitochondrial matrix that is thought to potentiate oxidative damage observed in FA. Therefore, the maintenance of mitochondrial homeostasis is crucial in the progression of neuro-degenerative conditions, particularly in FA. In this review, vital mitochondrial homeostatic processes and their roles in FA pathogenesis will be discussed. These include mitochondrial iron processing, mitochondrial dynamics (fusion and fission processes), mitophagy, mitochondrial biogenesis, mitochondrial energy production and calcium metabolism.

线粒体稳态对于维持健康的细胞功能和生存至关重要。线粒体功能障碍对神经退行性疾病的不利影响最近在帕金森病、阿尔茨海默病和亨廷顿病等人类疾病中得到了强调。弗里德赖希共济失调 (FA) 是另一种神经退行性疾病，也是心脏退行性疾病，其中线粒体功能障碍在疾病进展中起着至关重要的作用。线粒体蛋白 frataxin 的表达缺陷是 FA 的主要原因，它会导致全细胞和线粒体铁代谢的不利改变。这些区室中铁代谢的失调会导致线粒体基质中无机铁沉积物的积累，这被认为会增强 FA 中观察到的氧化损伤。因此，维持线粒体稳态对于神经退行性疾病（尤其是 FA）的进展至关重要。在这篇综述中，我们将讨论重要的线粒体稳态过程及其在 FA 发病机制中的作用。这些包括线粒体铁加工、线粒体动力学（融合和裂变过程）、线粒体自噬、线粒体生物发生、线粒体能量产生和钙代谢。