Organization and regulation of mitochondrial protein synthesis
Open accessAbstract
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.
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The shift from transcriptional control to posttranscriptional regulation is evolutionarily advanced compared to prokaryotic ancestors that lack PPR proteins.
“The shift from transcriptional control to posttranscriptional regulation is considered evolutionarily advanced compared to prokaryotic ancestors which do not contain PPR proteins (Ott et al. 2016; Siira et al. 2017; Seshadri et al. 2020).”
From <i>Candida albicans</i> PPR proteins are required for the expression of respiratory Complex I subunits (2024) · cites this paper as Ott et al. 2016
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Self-citationLack of secondary structure characterizes the 5′ ends of mammalian mitochondrial mRNAs
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Functional Interactions between Yeast Mitochondrial Ribosomes and mRNA 5′ Untranslated Leaders
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5S rRNA: Structure and Function from Head to Toe
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RNA degradation in yeast and human mitochondria.
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Mitochondria and cardiovascular diseases-from pathophysiology to treatment
Siasos, G., Tsigkou, V., Kosmopoulos, M., Theodosiadis, D., Simantiris, S., Tagkou, N. M., Tsimpiktsioglou, A., Stampouloglou, P. K., Oikonomou, E., Mourouzis, K., et al. (2018) · Annals of Translational Medicine · doi:10.21037/atm.2018.06.21 · Cited by 245 · see citing works
Coupling of import and assembly pathways in mitochondrial protein biogenesis
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Molecular connectivity of mitochondrial gene expression and OXPHOS biogenesis
Singh, A. P., Salvatori, R., Aftab, W., Kohler, A., Carlström, A., Forne, I., Imhof, A., & Ott, M. (2020) · Molecular Cell · doi:10.1016/j.molcel.2020.07.024 · Cited by 65 · see citing works
Self-citationSensing the Stress: A Role for the UPRmt and UPRam in the Quality Control of Mitochondria
Callegari, S., & Dennerlein, S. (2018) · Frontiers in Cell and Developmental Biology · doi:10.3389/fcell.2018.00031 · Cited by 58 · see citing works
Molecular wiring of a mitochondrial translational feedback loop
Salvatori, R., Kehrein, K., Singh, A. P., Aftab, W., Möller-Hergt, B. V., Forne, I., Imhof, A., & Ott, M. (2020) · Molecular Cell · doi:10.1016/j.molcel.2019.11.019 · Cited by 34 · see citing works
Self-citationWenda, J. M., Drzewicka, K., Mulica, P., Tetaud, E., di Rago, J. P., Golik, P., & Łabędzka-Dmoch, K. (2024) · GENETICS · doi:10.1093/genetics/iyae124 · Cited by 2 · see citing works
- Authors
- Ott, M., Amunts, A., & Brown, A.
- Affiliations
- Stockholm University (SE)
- MRC Laboratory of Molecular Biology (GB)
- Venue
- Annual Review of Biochemistry
- Publisher
- Annual Reviews
- Published
- 2016-06-02
- Volume
- 85
- Issue
- 1
- Pages
- 77-101
- ISSN
- 0066-4154 (ISSN-L)
- 1545-4509
- OpenAlex
- W2192985956
- Cited by
- 282
- References
- 154
- Citations by year
- 2026: 21 · 2025: 18 · 2024: 20 · 2023: 22 · 2022: 30 · 2021: 33 · 2020: 37 · 2019: 25 · 2018: 37 · 2017: 20 · 2016: 19
- FWCI
- 3.04
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Annual Review of Biochemistry (publishedVersion)
- PubMed (publishedVersion)
- Funders
- Wellcome Trust (096570/Z/11/Z)
- Stiftelsen för Strategisk Forskning
- Knut och Alice Wallenbergs Stiftelse
- Vetenskapsrådet
- Stockholms Universitet
- Medical Research Council
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA and protein synthesis mechanisms
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › RNA modifications and cancer
- Keywords
- mitochondrial translation, mitochondrial ribosome, mitochondrial genome, oxidative phosphorylation complexes, translational regulation, nuclear-encoded subunits, endosymbiotic origin of mitochondria
- MeSH terms
- Mitochondrial Ribosomes, Animals, Organelle Biogenesis, DNA, Mitochondrial, Biological Evolution, Gene Expression Regulation, Humans, Mitochondria, Models, Molecular, Oxidative Phosphorylation, Protein Biosynthesis, Signal Transduction, Mitochondrial Proteins, Mitochondrial Membranes, Genome, Mitochondrial
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