iMM Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisboa, Portugal.
iMM Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, 1649-028 Lisboa, Portugal.
Biochem Biophys Res Commun. 2018 May 27;500(1):45-50. doi: 10.1016/j.bbrc.2017.06.096. Epub 2017 Jun 21.
Mitochondria have a pivotal role in the maintenance of cell homeostasis and survival. Mitochondria are involved in processes such as ATP production, reactive oxygen species production, apoptosis induction, calcium homeostasis and protein degradation. Thus, mechanisms that regulate the intrinsic quality of mitochondria have a crucial role in dictating overall cell fate. The importance of these well-regulated mechanisms is highlighted in disease scenarios such as neurodegeneration, cancer and neuromuscular atrophy. How mitochondria senses and regulates their intrinsic quality control, and consequently cell survival, is still not fully understood. In this review, we discuss the pathways that are at present considered as state-of-the-art for mitochondria quality control regulation, and highlight a mitochondrial protein-PINK1-that has revealed to act as a mitochondrial gatekeeper able to sense the presence of healthy or damaged mitochondria.
线粒体在维持细胞内稳态和生存方面起着关键作用。线粒体参与了许多过程，如 ATP 生成、活性氧物种生成、细胞凋亡诱导、钙稳态和蛋白质降解。因此，调节线粒体内在质量的机制在决定细胞整体命运方面起着至关重要的作用。这些调控良好的机制在神经退行性疾病、癌症和神经肌肉萎缩等疾病中显得尤为重要。线粒体如何感知和调节其内在的质量控制，以及细胞的存活，目前还不完全清楚。在这篇综述中，我们讨论了目前被认为是线粒体质量控制调节的最新途径，并强调了一种线粒体蛋白-PINK1-它被证明可以作为线粒体的守门员，能够感知健康或受损线粒体的存在。
