title: Mitochondrial protein translocases for survival and wellbeing.
abstract: Mitochondria are involved in many essential cellular activities. These broad functions explicate the need for the well-orchestrated biogenesis of mitochondrial proteins to avoid death and pathological consequences, both in unicellular and more complex organisms. Yeast as a model organism has been pivotal in identifying components and mechanisms that drive the transport and sorting of nuclear-encoded mitochondrial proteins. The machinery components that are involved in the import of mitochondrial proteins are generally evolutionarily conserved within the eukaryotic kingdom. However, topological and functional differences have been observed. We review the similarities and differences in mitochondrial translocases from yeast to human. Additionally, we provide a systematic overview of the contribution of mitochondrial import machineries to human pathologies, including cancer, mitochondrial diseases, and neurodegeneration.
authors.affiliation: Laboratory of Mitochondrial Biogenesis, International Institute of Molecular and Cell Biology, Warsaw 02-109, Poland.
authors.forename: Anna Magdalena
authors.initials: AM
authors.lastname: Sokol
authors.affiliation: Laboratory of Mitochondrial Biogenesis, International Institute of Molecular and Cell Biology, Warsaw 02-109, Poland.
authors.forename: Malgorzata Eliza
authors.initials: ME
authors.lastname: Sztolsztener
authors.affiliation: Laboratory of Mitochondrial Biogenesis, International Institute of Molecular and Cell Biology, Warsaw 02-109, Poland.
authors.forename: Michal
authors.initials: M
authors.lastname: Wasilewski
authors.affiliation: Department of Microbiology, Monash University, Melbourne, Victoria 3800, Australia; Victorian Bioinformatics Consortium, Monash University, Melbourne, Victoria 3800, Australia.
authors.forename: Eva
authors.initials: E
authors.lastname: Heinz
authors.affiliation: Laboratory of Mitochondrial Biogenesis, International Institute of Molecular and Cell Biology, Warsaw 02-109, Poland. Electronic address: achacinska@iimcb.gov.pl.
authors.forename: Agnieszka
authors.initials: A
authors.lastname: Chacinska
mesh_terms: D000818:Animals; D006801:Humans; D008928:Mitochondria / Q000201:enzymology / Q000378:metabolism* / Q000473:pathology; D028361:Mitochondrial Diseases / Q000235:genetics / Q000378:metabolism*; D033681:Mitochondrial Membrane Transport Proteins / Q000235:genetics / Q000378:metabolism*; D019636:Neurodegenerative Diseases / Q000235:genetics / Q000378:metabolism*; D021381:Protein Transport; D012441:Saccharomyces cerevisiae / Q000201:enzymology / Q000235:genetics / Q000378:metabolism; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics / Q000378:metabolism
chemical_list: D033681:Mitochondrial Membrane Transport Proteins; D029701:Saccharomyces cerevisiae Proteins
keywords: Cancer; HIF1α; Mitochondrial disease; Neurodegeneration; Protein biogenesis and import; p53