Thank you for visiting nature.com. You are using a browser version with limited support for CSS. To obtain
the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in
Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles
and JavaScript.
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.
Mary Herbert and Doug Turnbull discuss how the transmission of pathogenic mutations in mitochondrial DNA may be prevented by reproductive techniques designed to replace the mitochondria in eggs from affected women.
Navdeep Chandel highlights a study published in 1996 that — by showing that mitochondria can release cytochromecto initiate apoptosis — changed his view of the role of mitochondria in physiology.
Basal mitophagy can occur independently of the kinase PINK1 in mammalian organisms, calling for further studies to elucidate the mechanisms of mitophagyin vivo.