Interaction and regulation of the mitochondrial proteome - in health and disease.

Introduction: Mitochondria contain multiple pathways including energy metabolism and several signaling and synthetic pathways. Mitochondrial proteomics is highly valuable for studying diseases including inherited metabolic disorders, complex and common disorders like neurodegeneration, diabetes and cancer, since they all to some degree have mitochondrial underpinnings.

Areas Covered: The main mitochondrial functions and pathways are outlined and systematic protein lists are presented. In addition to the main energy metabolic pathways are; iron-sulfur cluster synthesis, one carbon metabolism, catabolism of hydrogen sulfide, kynurenines and reactive oxygen species (ROS), and others, described with the aim of laying a foundation for systematic mitochondrial pathway analysis based on proteomics data. The links of the proteins and pathways to functional effects and diseases are discussed. The disease examples are focussed on inherited metabolic disorders, cancer, neurological and cardiovascular disorders.

Expert Opinion: To elucidate the roles of mitochondria in health and disease, there is a need for comprehensive proteomics analyses with stringent, systematic data treatment for proper interpretation of mitochondrial pathway data. In that way comprehensive hypothesis-based research can be performed based on proteomics data.