Mitochondrial Respiratory Supercomplexes in Physiology and Diseases

In eukaryotic cells, mitochondria play the fundamental role of ATP production during the process of oxidative phosphorylation (OXPHOS). However, these cytosolic organelles also have several other important physiological functions, including sugar and fatty acid catabo- lism, amino acid metabolism, buffering of the cytosolic calcium concentration (Rizzuto et al., 2012), regulation and execution of different types of cell death (Galluzzi et al., 2012) and arrangement of adaptive responses to perturbations of intracellular homeostasis (Liesa and Shirihai, 2013). Furthermore, mitochondria are able to discharge a range of intracel- lular signals including reactive oxygen species (ROS), mitochondrial DNA (mtDNA) and specific proteins, thus operating as fundamental hubs of a wide array of signalling pathways (Galluzzi et al., 2012).