Mitochondria are the sites of oxygen respiration, oxidative phosphorylation, and energy production. More than just the “powerhouse of the cell,” they also mediate lipid metabolism, cytosolic calcium buffering, cofactor synthesis, apoptosis, and necrosis. They are incredibly dynamic organelles that undergo fission, fusion, intracellular motility (transport), and multiple forms of quality control. Although mitochondria possess their own genome, most of the proteins that reside in mitochondria are encoded by the nuclear genome and must be imported across the double membrane structure of the mitochondria. Dysfunctional mitochondria play a role in multiple human diseases, including ischemic stroke, Parkinson’s Disease, Alzheimer’s Disease, ALS, Leigh Syndrome, optic atrophy, and cancer. Mitochondrial function and regulation are going to be key elements for addressing these diseases.