title: Quality control of mitochondrial proteostasis.
abstract: A decline in mitochondrial activity has been associated with aging and is a hallmark of many neurological diseases. Surveillance mechanisms acting at the molecular, organellar, and cellular level monitor mitochondrial integrity and ensure the maintenance of mitochondrial proteostasis. Here we will review the central role of mitochondrial chaperones and proteases, the cytosolic ubiquitin-proteasome system, and the mitochondrial unfolded response in this interconnected quality control network, highlighting the dual function of some proteases in protein quality control within the organelle and for the regulation of mitochondrial fusion and mitophagy.
authors.affiliation: Institute for Genetics, Center for Molecular Medicine (CMMC), Cologne Excellence Cluster on Cellular Stress Responses in Aging-Associated Diseases (CECAD), University of Cologne, 50674 Cologne, Germany.
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Baker
authors.forename: Takashi
authors.initials: T
authors.lastname: Tatsuta
authors.forename: Thomas
authors.initials: T
authors.lastname: Langer
mesh_terms: D000255:Adenosine Triphosphate / Q000378:metabolism; D000818:Animals; D016922:Cellular Senescence; D020558:GTP Phosphohydrolases / Q000378:metabolism / Q000502:physiology; D006706:Homeostasis; D006801:Humans; D008928:Mitochondria / Q000378:metabolism / Q000502:physiology* / Q000648:ultrastructure; D024101:Mitochondrial Proteins / Q000378:metabolism* / Q000502:physiology; D008954:Models, Biological; D018832:Molecular Chaperones / Q000378:metabolism* / Q000502:physiology; D010447:Peptide Hydrolases / Q000378:metabolism / Q000502:physiology; D046988:Proteasome Endopeptidase Complex / Q000378:metabolism / Q000502:physiology; D017510:Protein Folding; D025801:Ubiquitin / Q000378:metabolism
chemical_list: D024101:Mitochondrial Proteins; D018832:Molecular Chaperones; D025801:Ubiquitin; D000255:Adenosine Triphosphate; D010447:Peptide Hydrolases; D046988:Proteasome Endopeptidase Complex; D020558:GTP Phosphohydrolases