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Aging/genetics/*metabolism/pathology
Animals
Genome, Human
Humans
Metabolic Syndrome/*enzymology/genetics/pathology
Mitochondria/*enzymology/genetics/pathology
Mitochondrial Proteins/genetics/*metabolism
Neoplasm Proteins/genetics/*metabolism
Neoplasms/*enzymology/genetics/pathology
Neurodegenerative Diseases/*enzymology/genetics/pathology
Peptide Hydrolases/genetics/*metabolism
Proteolysis
Abstract:
Recent advances in mitochondrial biology have revealed the high diversity and complexity of proteolytic enzymes that regulate mitochondrial function. We have classified mitochondrial proteases, or mitoproteases, on the basis of their function and location, and defined the human mitochondrial degradome as the complete set of mitoproteases that are encoded by the human genome. In addition to their nonspecific degradative functions, mitoproteases perform highly regulated proteolytic reactions that are important in mitochondrial function, integrity and homeostasis. These include protein synthesis, quality control, mitochondrial biogenesis and dynamics, mitophagy and apoptosis. Impaired or dysregulated function of mitoproteases is associated with ageing and with many pathological conditions such as neurodegenerative disorders, metabolic syndromes and cancer. A better understanding of the mitochondrial proteolytic landscape and its modulation may contribute to improving human lifespan and 'healthspan'.
