Conflict of interest: AMKC is a cofounder of, is a stockholder of, and serves on the Scientific Advisory Board for Proterris, Inc., which develops therapeutic uses for carbon monoxide. AMKC also has a use patent (US 7,678,390) on CO. AMKC served as a consultant for Teva Pharmaceuticals in July 2018. The spouse of MEC is a cofounder of, is a shareholder of, and serves on the Scientific Advisory Board of Proterris, Inc.

Mitochondria are well-known as organelles responsible for the maintenance of cellular bioenergetics through the production of ATP. While oxidative phosphorylation may be their most important function, mitochondria are also integral for the synthesis of metabolic precursors, calcium regulation, the production of reactive oxygen species, immune signaling, and apoptosis. Considering the breadth of their responsibilities, mitochondria are fundamental for cellular metabolism and homeostasis. Appreciating this significance, translational medicine has begun to investigate how mitochondrial dysfunction can represent a harbinger of disease. In this review, we provide a detailed overview of mitochondrial metabolism, cellular bioenergetics, mitochondrial dynamics, autophagy, mitochondrial damage-associated molecular patterns, mitochondria-mediated cell-death pathways, and how mitochondrial dysfunction at any of these levels is associated with disease pathogenesis. Mitochondria-dependent pathways may thereby represent an attractive therapeutic target for ameliorating human disease.