Physiological and Pathological Functions of Mitochondrial Proteases

Mitoproteases display an essential role in the preservation of mitochondrial homeostasis under regular and stress conditions. These enzymes perform tightly regulated proteolytic reactions by which they participate in mitochondrial protein trafficking, processing and activation of proteins, protein quality control, regulation of mitochondrial biogenesis, control of mitochondrial dynamics, mitophagy, and apoptosis. In this chapter, we have revised the physiological functions of the intrinsic mitochondrial proteases, analyzing their roles in the different compartments of this organelle and their connection to human pathology, primarily cancer, neurodegenerative disorders, and multisystemic diseases.