title: Chatty Mitochondria: Keeping Balance in Cellular Protein Homeostasis.
abstract: Mitochondria are multifunctional cellular organelles that host many biochemical pathways including oxidative phosphorylation (OXPHOS). Defective mitochondria pose a threat to cellular homeostasis and compensatory responses exist to curtail the source of stress and/or its consequences. The mitochondrial proteome comprises proteins encoded by the nuclear and mitochondrial genomes. Disturbances in protein homeostasis may originate from mistargeting of nuclear encoded mitochondrial proteins. Defective protein import and accumulation of mistargeted proteins leads to stress that triggers translation alterations and proteasomal activation. These cytosolic pathways are complementary to the mitochondrial unfolded protein response (UPRmt) that aims to increase the capacity of protein quality control mechanisms inside mitochondria. They constitute putative targets for interventions aimed at increasing the fitness, stress resistance, and longevity of cells and organisms.
authors.affiliation: International Institute of Molecular and Cell Biology, Ks Trojdena 4, 02-109 Warsaw, Poland.
authors.forename: Ulrike
authors.initials: U
authors.lastname: Topf
authors.affiliation: International Institute of Molecular and Cell Biology, Ks Trojdena 4, 02-109 Warsaw, Poland.
authors.forename: Lidia
authors.initials: L
authors.lastname: Wrobel
authors.affiliation: International Institute of Molecular and Cell Biology, Ks Trojdena 4, 02-109 Warsaw, Poland. Electronic address: achacinska@iimcb.gov.pl.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chacinska
mesh_terms: D000818:Animals; D002477:Cells / Q000378:metabolism*; D006801:Humans; D008928:Mitochondria / Q000378:metabolism* / Q000473:pathology; D059748:Proteolysis; D000074702:Proteostasis / Q000235:genetics; D013312:Stress, Physiological; D014158:Transcription, Genetic
keywords: protein biogenesis; protein degradation; protein transport; stress response; translation; unfolded protein response