ArticleThe EMBO journal2019
Stress signaling and cellular proliferation reverse the effects of mitochondrial mistranslation.
Article in The EMBO journal, 2019. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 32 citations in OpenAlex.
- Systemic delivery of AAV-GFM1 corrects COXPD1 molecular alterations in Gfm1EMBO molecular medicine · 2026Article
- Mechanisms and disease relevance of mitochondrial translation in humans.Nature reviews. Molecular cell biology · 2026Review
- Specific SLC25 carriers regulate mitochondrial protein synthesis.Science advances · 2026Article
- TANGO2 binds crystallin alpha B and its loss causes desminopathy.Nature communications · 2025Article
- Haplotype variability in mitochondrial rRNA predisposes to metabolic syndrome.Communications biology · 2024Article
- Illuminating mitochondrial translation through mouse models.Human molecular genetics · 2024Review
- Quantitative subcellular reconstruction reveals a lipid mediated inter-organelle biogenesis network.Nature cell biology · 2024Article
- ATFS-1 counteracts mitochondrial DNA damage by promoting repair over transcription.Nature cell biology · 2023Article
- Copy number variation in tRNA isodecoder genes impairs mammalian development and balanced translation.Nature communications · 2023Article
- Structural analysis of mitochondrial rRNA gene variants identified in patients with deafness.Frontiers in physiology · 2023Article
- Our current understanding of the toxicity of altered mito-ribosomal fidelity during mitochondrial protein synthesis: What can it tell us about human disease?Frontiers in physiology · 2023Review
- Deleterious variants in CRLS1 lead to cardiolipin deficiency and cause an autosomal recessive multi-system mitochondrial disease.Human molecular genetics · 2022Article
- Organization and expression of the mammalian mitochondrial genome.Nature reviews. Genetics · 2022Review
- Mitochondrial rRNA Methylation by Mettl15 Contributes to the Exercise and Learning Capability in Mice.International journal of molecular sciences · 2022Article
- Mitochondrial mistranslation modulated by metabolic stress causes cardiovascular disease and reduced lifespan.Aging cell · 2021Article
- Mechanisms and regulation of protein synthesis in mitochondria.Nature reviews. Molecular cell biology · 2021Review
- Metabolic shift underlies recovery in reversible infantile respiratory chain deficiency.The EMBO journal · 2020Article
- Skeletal Phenotypes Due to Abnormalities in Mitochondrial Protein Homeostasis and Import.International journal of molecular sciences · 2020Review
- Intimate Relations-Mitochondria and Ageing.International journal of molecular sciences · 2020Review
- Structural basis of mitochondrial translation.eLife · 2020Article
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Authors and funding
18 authors at 4 institutions in 1 country.
Funding
Abstract
Translation fidelity is crucial for prokaryotes and eukaryotic nuclear-encoded proteins; however, little is known about the role of mistranslation in mitochondria and its potential effects on metabolism. We generated yeast and mouse models with error-prone and hyper-accurate mitochondrial translation, and found that translation rate is more important than translational accuracy for cell function in mammals. Specifically, we found that mitochondrial mistranslation causes reduced overall mitochondrial translation and respiratory complex assembly rates. In mammals, this effect is compensated for by increased mitochondrial protein stability and upregulation of the citric acid cycle. Moreover, this induced mitochondrial stress signaling, which enables the recovery of mitochondrial translation via mitochondrial biogenesis, telomerase expression, and cell proliferation, and thereby normalizes metabolism. Conversely, we show that increased fidelity of mitochondrial translation reduces the rate of protein synthesis without eliciting a mitochondrial stress response. Consequently, the rate of translation cannot be recovered and this leads to dilated cardiomyopathy in mice. In summary, our findings reveal mammalian-specific signaling pathways that respond to changes in the fidelity of mitochondrial protein synthesis and affect metabolism.
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