Department of Neurology, University of Miami School of Medicine, Miami, FL 33136, USA.
IUBMB Life. 2009 Nov;61(11):1051-62. doi: 10.1002/iub.261.
Mitochondria play a central role in cellular homeostasis. Hence, mitochondrial dysfunction resulting in impaired ATP supply is detrimental to cell viability and causes severe and often fatal disorders. Current treatment of these mitochondrial disorders is limited and mostly addresses the symptoms, but not the deficiency itself. Recent work in cell and animal models of mitochondrial disease shows that increased mitochondrial biogenesis can boost residual OXPHOS capacity and thereby prevent the bioenergetic crisis. In animal models, this strategy results in increased health and lifespan. Here, I review which mitochondrial processes are affected in known mitochondrial disorders and discuss why PGC-1alpha mediated mitochondrial biogenesis offers a promising venue for treatment of mitochondrial disorders.
线粒体在细胞内稳态中起着核心作用。因此，线粒体功能障碍导致 ATP 供应受损，对细胞活力有害，并导致严重且常致命的疾病。目前对这些线粒体疾病的治疗有限，主要针对症状，而不是缺乏本身。最近在线粒体疾病的细胞和动物模型中的研究表明，增加线粒体生物发生可以提高剩余 OXPHOS 能力，从而防止生物能量危机。在动物模型中，这种策略会增加健康和寿命。在这里，我回顾了已知的线粒体疾病中受影响的线粒体过程，并讨论了为什么 PGC-1alpha 介导的线粒体生物发生为治疗线粒体疾病提供了一个有前途的途径。
