title: Mitochondrial quality control pathways as determinants of metabolic health.
abstract: Mitochondrial function is key for maintaining cellular health, while mitochondrial failure is associated with various pathologies, including inherited metabolic disorders and age-related diseases. In order to maintain mitochondrial quality, several pathways of mitochondrial quality control have evolved. These systems monitor mitochondrial integrity through antioxidants, DNA repair systems, and chaperones and proteases involved in the mitochondrial unfolded protein response. Additional regulation of mitochondrial function involves dynamic exchange of components through mitochondrial fusion and fission. Sustained stress induces a selective autophagy - termed mitophagy - and ultimately leads to apoptosis. Together, these systems form a network that acts on the molecular, organellar, and cellular level. In this review, we highlight how these systems are regulated in an integrated context- and time-dependent network of mitochondrial quality control that is implicated in healthy aging.
authors.affiliation: Laboratory Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Ntsiki M
authors.initials: NM
authors.lastname: Held
authors.affiliation: Laboratory Genetic Metabolic Diseases, Academic Medical Center, Amsterdam, the Netherlands.
authors.forename: Riekelt H
authors.initials: RH
authors.lastname: Houtkooper
mesh_terms: D000818:Animals; D017209:Apoptosis; D006801:Humans; D008928:Mitochondria / Q000502:physiology*; D063154:Mitochondrial Dynamics; D024101:Mitochondrial Proteins / Q000378:metabolism; D063306:Mitophagy; D053140:Organelle Shape; D059748:Proteolysis; D017382:Reactive Oxygen Species / Q000378:metabolism*; D056811:Unfolded Protein Response
chemical_list: D024101:Mitochondrial Proteins; D017382:Reactive Oxygen Species
keywords: ROS; fission; fusion; mitochondrial dynamics; mitochondrial quality control; mitohormesis; mitophagy