7a5i Citations

Elongational stalling activates mitoribosome-associated quality control.

Science 370 1105-1110 (2020)
Related entries: 7a5f, 7a5g, 7a5h, 7a5j, 7a5k

Cited: 48 times
EuropePMC logo PMID: 33243891

Abstract

The human mitochondrial ribosome (mitoribosome) and associated proteins regulate the synthesis of 13 essential subunits of the oxidative phosphorylation complexes. We report the discovery of a mitoribosome-associated quality control pathway that responds to interruptions during elongation, and we present structures at 3.1- to 3.3-angstrom resolution of mitoribosomal large subunits trapped during ribosome rescue. Release factor homolog C12orf65 (mtRF-R) and RNA binding protein C6orf203 (MTRES1) eject the nascent chain and peptidyl transfer RNA (tRNA), respectively, from stalled ribosomes. Recruitment of mitoribosome biogenesis factors to these quality control intermediates suggests additional roles for these factors during mitoribosome rescue. We also report related cryo-electron microscopy structures (3.7 to 4.4 angstrom resolution) of elongating mitoribosomes bound to tRNAs, nascent polypeptides, the guanosine triphosphatase elongation factors mtEF-Tu and mtEF-G1, and the Oxa1L translocase.

Reviews - 7a5i mentioned but not cited (1)

  1. Maintaining mitochondrial ribosome function: The role of ribosome rescue and recycling factors. Nadler F, Lavdovskaia E, Richter-Dennerlein R. RNA Biol 19 117-131 (2022)


Reviews citing this publication (16)

  1. Mechanisms and regulation of protein synthesis in mitochondria. Kummer E, Ban N. Nat Rev Mol Cell Biol 22 307-325 (2021)
  2. Organization and expression of the mammalian mitochondrial genome. Rackham O, Filipovska A. Nat Rev Genet 23 606-623 (2022)
  3. Mitochondrial Protein Translation: Emerging Roles and Clinical Significance in Disease. Wang F, Zhang D, Zhang D, Li P, Gao Y. Front Cell Dev Biol 9 675465 (2021)
  4. Detecting and Rescuing Stalled Ribosomes. Yip MCJ, Shao S. Trends Biochem Sci 46 731-743 (2021)
  5. Ribosome Rescue Pathways in Bacteria. Müller C, Crowe-McAuliffe C, Wilson DN. Front Microbiol 12 652980 (2021)
  6. Human Mitoribosome Biogenesis and Its Emerging Links to Disease. Lopez Sanchez MIG, Krüger A, Shiriaev DI, Liu Y, Rorbach J. Int J Mol Sci 22 3827 (2021)
  7. Human Mitochondrial RNA Processing and Modifications: Overview. Jedynak-Slyvka M, Jabczynska A, Szczesny RJ. Int J Mol Sci 22 7999 (2021)
  8. Our current understanding of the toxicity of altered mito-ribosomal fidelity during mitochondrial protein synthesis: What can it tell us about human disease? Vila-Sanjurjo A, Mallo N, Atkins JF, Elson JL, Smith PM. Front Physiol 14 1082953 (2023)
  9. Bacterial Ribosome Rescue Systems. Kurita D, Himeno H. Microorganisms 10 372 (2022)
  10. Diversity and Similarity of Termination and Ribosome Rescue in Bacterial, Mitochondrial, and Cytoplasmic Translation. Korostelev AA. Biochemistry (Mosc) 86 1107-1121 (2021)
  11. RNA binding protein: coordinated expression between the nuclear and mitochondrial genomes in tumors. Ma J, Sun L, Gao W, Li Y, Dong D. J Transl Med 21 512 (2023)
  12. Mitochondrial protein synthesis quality control. Koludarova L, Battersby BJ. Hum Mol Genet 33 R53-R60 (2024)
  13. Navigating the landscape of mitochondrial-ER communication in health and disease. Ronayne CT, Latorre-Muro P. Front Mol Biosci 11 1356500 (2024)
  14. Decoding the Enigma: Translation Termination in Human Mitochondria. Krüger A, Kovalchuk D, Shiriaev D, Rorbach J. Hum Mol Genet 33 R42-R46 (2024)
  15. Illuminating mitochondrial translation through mouse models. Hughes LA, Rackham O, Filipovska A. Hum Mol Genet 33 R61-R79 (2024)
  16. Molecular pathways in mitochondrial disorders due to a defective mitochondrial protein synthesis. Antolínez-Fernández Á, Esteban-Ramos P, Fernández-Moreno MÁ, Clemente P. Front Cell Dev Biol 12 1410245 (2024)

Articles citing this publication (31)