East Daniel A, Campanella Michelangelo
Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, United Kingdom; Regina Elena-National Cancer Institute, 00144 Rome, Italy.
Department of Comparative Biomedical Sciences, The Royal Veterinary College, University of London, United Kingdom; University College London Consortium for Mitochondrial Research, Royal College Street, NW1 0TU London, United Kingdom; Department of Biology, University of Rome Tor Vergata, 00133 Rome, Italy; Regina Elena-National Cancer Institute, 00144 Rome, Italy.
Int J Biochem Cell Biol. 2016 Oct;79:382-387. doi: 10.1016/j.biocel.2016.08.019. Epub 2016 Aug 30.
Mitochondria are the foremost producers of the cellular energy currency ATP. They are also a significant source of reactive oxygen species and an important buffer of intracellular calcium. Mitochondrial retrograde signals regulate energy homeostasis and pro-survival elements whereas anterograde stimuli can trigger programmed cell death. Maintenance of a healthy, functional mitochondria network is therefore essential, and several mechanisms of mitochondrial quality control have been described. Mitochondrial dysfunction is linked to several neurodegenerative conditions including Parkinson, and Huntingdon diseases as well as Amyotrophic lateral sclerosis. Understanding the mechanisms governing mitochondrial quality control may reveal novel strategies for pharmacological intervention and disease therapy.
线粒体是细胞能量货币ATP的主要生产者。它们也是活性氧的重要来源和细胞内钙的重要缓冲剂。线粒体逆行信号调节能量稳态和促生存元件,而顺行刺激可触发程序性细胞死亡。因此,维持健康、功能正常的线粒体网络至关重要,并且已经描述了几种线粒体质量控制机制。线粒体功能障碍与包括帕金森病、亨廷顿病以及肌萎缩侧索硬化症在内的几种神经退行性疾病有关。了解线粒体质量控制的机制可能会揭示药物干预和疾病治疗的新策略。