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, pro
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Physiological and Pathological Functions of Mitochondrial Proteases Clea Bárcena, Pablo Mayoral, Pedro M. Quirós, and Carlos López-Otín
Abstract
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. Keywords
Mitochondria • Mitoproteases • Mitochondrial dynamics • Cancer • Aging • Neurodegenerative disorders
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Introduction
Due to their prokaryotic origin, mitochondria possess some structural characteristics that make them remarkably different from other organelles of eukaryotic cells. They have a double membrane with an intermembrane space, being the inner membrane expanded by the formation of numerous foldings named cristae. Enclosed among these cristae, we can find the mitochondrial matrix, where several copies of mitochondrial DNA genome are contained. Mitochondria play essential biological C. Bárcena • P. Mayoral • P.M. Quirós • C. López-Otín (*) Departamento de Bioquímica y Biología Molecular, Facultad de Medicina, Instituto Universitario de Oncología (IUOPA), Universidad de Oviedo, 33006 Oviedo, Spain e-mail: [email protected] © Springer Nature Singapore Pte Ltd. 2017 S. Chakraborti, N.S. Dhalla (eds.), Proteases in Physiology and Pathology, DOI 10.1007/978-981-10-2513-6_1
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functions, fundamental for the generation of most of the cell supply of adenosine triphosphate (ATP), the source of chemical energy within the cells. The complex structure and the relevance of the mitochondrial function within the organisms justify the need to set an organization of the molecules that collaborate in the maintenance of the assembly and function of this energy machine, among which proteases are gaining an increasing attention. Proteases have been considered for many years just as performers of the catabolic reactions in the organisms; however, we are currently beholding a surprising and unexpected increment in the studies of these enzymes, positioning them as multifunctional molecules carrying essential functions in health and disease [1–3]. The study of proteases contained in the mitochondria, named mitoproteases, has also experienced an exciting expansion. Until very recently, they were seen only as members of the quality control system of mitochondria, in charge of the degradation of misfolded and damaged proteins or being responsible for the processing of proteins imported from cytosol into mitochondria [4]. Nevertheless, in recent years, we have witn
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