Mitochondrial biogenesis is indispensable for organismal homeostasis. The semi-autonomous nature of mitochondria renders their biogenesis rather complex, as it requires the contribution of the nucleus, the cytoplasm and the organelle itself. Recently, several transcription regulators, RNA binding proteins and outer mitochondrial membrane (OMM) components have been implicated in the coordination of the process. Both the expression and the abundance of several of these factors are altered during ageing, and their impairment can have diverse, yet principally detrimental, effects on lifespan. These findings converge on the notion that mitochondrial biogenesis is an age-modulated process that, when perturbed, compromises survival. Notably, core brain functions are dependent on mitochondrial metabolite availability. Indeed, emerging evidence indicates that mitochondrial biogenesis regulators play important roles in the onset and progression of severe neurodegenerative syndromes such as AD, PD and HD. These devastating human pathologies remain incurable to date. A better understanding of the mechanisms that govern mitochondrial biogenesis could facilitate the development of effective pharmaceutical interventions against these diseases.

线粒体生物发生对于机体稳态是必不可少的。线粒体的半自主性质使其生物发生相当复杂，因为它需要细胞核、细胞质和细胞器本身的贡献。最近，一些转录调节因子、RNA 结合蛋白和线粒体外膜 (OMM) 成分与该过程的协调有关。这些因素中的一些因素的表达和丰度在衰老过程中都会发生变化，它们的损害会对寿命产生多种但主要是有害的影响。这些发现一致认为线粒体生物发生是一个受年龄调节的过程，当受到干扰时，会危及生存。值得注意的是，核心大脑功能取决于线粒体代谢物的可用性。的确，新出现的证据表明，线粒体生物发生调节因子在严重神经退行性疾病（如 AD、PD 和 HD）的发生和进展中发挥重要作用。迄今为止，这些毁灭性的人类病理仍然无法治愈。更好地了解控制线粒体生物发生的机制可以促进针对这些疾病的有效药物干预的开发。