Nature Reviews Molecular Cell Biology | Review Article
The integrity of mitochondria, which are the main producers of cellular energy, is often compromised in disease and ageing. This focus highlights several aspects of the regulation of mitochondrial function and homeostasis and their links to disease and ageing. It comprises Reviews that discuss mechanisms of mitophagy, which removes damaged mitochondria, the mitochondrial unfolded protein response, which is a stress response that promotes the recovery of a functional mitochondrial network, and the recent insights into how AMPK integrates energy metabolism and mitochondrial dynamics. The focus also includes a Review of the principles underlying human mitochondrial diseases and a Comment on the progress in mitochondrial replacement therapies, which prevent mother-to-offspring disease transmission.