title: Organization and Regulation of Mitochondrial Protein Synthesis.
abstract: Mitochondria are essential organelles of endosymbiotic origin that are responsible for oxidative phosphorylation within eukaryotic cells. Independent evolution between species has generated mitochondrial genomes that are extremely diverse, with the composition of the vestigial genome determining their translational requirements. Typically, translation within mitochondria is restricted to a few key subunits of the oxidative phosphorylation complexes that are synthesized by dedicated ribosomes (mitoribosomes). The dramatically rearranged mitochondrial genomes, the limited set of transcripts, and the need for the synthesized proteins to coassemble with nuclear-encoded subunits have had substantial consequences for the translation machinery. Recent high-resolution cryo-electron microscopy has revealed the effect of coevolution on the mitoribosome with the mitochondrial genome. In this review, we place the new structural information in the context of the molecular mechanisms of mitochondrial translation and focus on the novel ways protein synthesis is organized and regulated in mitochondria.
authors.affiliation: Department of Biochemistry and Biophysics, Stockholm University, SE-106 91 Stockholm, Sweden; email: martin.ott@dbb.su.se.
authors.forename: Martin
authors.initials: M
authors.lastname: Ott
authors.affiliation: Department of Biochemistry and Biophysics, Stockholm University, SE-106 91 Stockholm, Sweden; email: martin.ott@dbb.su.se.|Science for Life Laboratory, Stockholm University, SE-171 21 Solna, Sweden; email: amunts@scilifelab.se.
authors.forename: Alexey
authors.initials: A
authors.lastname: Amunts
authors.affiliation: Laboratory of Molecular Biology, Medical Research Council, Cambridge CB2 0QH, United Kingdom; email: abrown1@mrc-lmb.cam.ac.uk.
authors.forename: Alan
authors.initials: A
authors.lastname: Brown
mesh_terms: D000818:Animals; D005075:Biological Evolution; D004272:DNA, Mitochondrial / Q000235:genetics* / Q000378:metabolism; D005786:Gene Expression Regulation; D054629:Genome, Mitochondrial; D006801:Humans; D008928:Mitochondria / Q000235:genetics* / Q000378:metabolism; D051336:Mitochondrial Membranes / Q000378:metabolism; D024101:Mitochondrial Proteins / Q000235:genetics* / Q000378:metabolism; D000069396:Mitochondrial Ribosomes / Q000737:chemistry / Q000378:metabolism; D008958:Models, Molecular; D001678:Organelle Biogenesis; D010085:Oxidative Phosphorylation; D014176:Protein Biosynthesis; D015398:Signal Transduction
chemical_list: D004272:DNA, Mitochondrial; D024101:Mitochondrial Proteins
keywords: biogenesis; evolution; mitochondria; mitoribosome; translation