title: Oxidative stress and mitochondrial protein quality control in aging.
abstract: Mitochondrial protein quality control incorporates an elaborate network of chaperones and proteases that survey the organelle for misfolded or unfolded proteins and toxic aggregates. Repair of misfolded or aggregated protein and proteolytic removal of irreversibly damaged proteins are carried out by the mitochondrial protein quality control system. Initial maturation and folding of the nuclear or mitochondrial-encoded mitochondrial proteins are mediated by processing peptidases and chaperones that interact with the protein translocation machinery. Mitochondrial proteins are subjected to cumulative oxidative damage. Thus, impairment of quality control processes may cause mitochondrial dysfunction. Aging has been associated with a marked decline in the effectiveness of mitochondrial protein quality control. Here, we present an overview of the chaperones and proteases involved in the initial folding and maturation of new, incoming precursor molecules, and the subsequent repair and removal of oxidized aggregated proteins. In addition, we highlight the link between mitochondrial protein quality control mechanisms and the aging process. This article is part of a Special Issue entitled: Posttranslational Protein modifications in biology and Medicine.
authors.affiliation: Institute of Molecular Biology and Biotechnology, Foundation for Research and Technology-Hellas, Heraklion 71110, Crete, Greece.
authors.forename: Eirini
authors.initials: E
authors.lastname: Lionaki
authors.forename: Nektarios
authors.initials: N
authors.lastname: Tavernarakis
mesh_terms: D000375:Aging / Q000378:metabolism* / Q000473:pathology; D000818:Animals; D006801:Humans; D024101:Mitochondrial Proteins / Q000378:metabolism*; D018832:Molecular Chaperones / Q000378:metabolism; D018384:Oxidative Stress; D010447:Peptide Hydrolases / Q000378:metabolism; D017510:Protein Folding; D021381:Protein Transport; D059748:Proteolysis
chemical_list: D024101:Mitochondrial Proteins; D018832:Molecular Chaperones; D010447:Peptide Hydrolases
keywords: AAA; ATPases Associated with various cellular activities; Chaperone; Clp; Filament forming temperature sensitive; FtsH; Hsp; IMP; IMS; MPP; MTS; Mitochondria; Oxidative stress; PAM; Peptidase; Protein quality control; Protein translocation; RNA interference; RNAi; TCA; TIM; TOM; TPR; UPR(mt); caseinolytic protease; heat-shock protein; inner membrane peptidase; intermembrane space; mitochondrial DNA; mitochondrial processing peptidase; mitochondrial protein quality control; mitochondrial targeting signal; mitochondrial unfolded protein response.; mtDNA; mtPQC; presequence translocase associated motor; tetratricopeptide repeat; translocase of the inner membrane; translocase of the outer membrane; tricarboxylic acid