8oin Citations

Molecular basis of translation termination at noncanonical stop codons in human mitochondria.

Abstract

The genetic code that specifies the identity of amino acids incorporated into proteins during protein synthesis is almost universally conserved. Mitochondrial genomes feature deviations from the standard genetic code, including the reassignment of two arginine codons to stop codons. The protein required for translation termination at these noncanonical stop codons to release the newly synthesized polypeptides is not currently known. In this study, we used gene editing and ribosomal profiling in combination with cryo-electron microscopy to establish that mitochondrial release factor 1 (mtRF1) detects noncanonical stop codons in human mitochondria by a previously unknown mechanism of codon recognition. We discovered that binding of mtRF1 to the decoding center of the ribosome stabilizes a highly unusual conformation in the messenger RNA in which the ribosomal RNA participates in specific recognition of the noncanonical stop codons.

Reviews citing this publication (2)

  1. Decoding the Enigma: Translation Termination in Human Mitochondria. Krüger A, Kovalchuk D, Shiriaev D, Rorbach J. Hum Mol Genet 33 R42-R46 (2024)
  2. Illuminating mitochondrial translation through mouse models. Hughes LA, Rackham O, Filipovska A. Hum Mol Genet 33 R61-R79 (2024)

Articles citing this publication (4)

  1. Structural insights into the role of GTPBP10 in the RNA maturation of the mitoribosome. Nguyen TG, Ritter C, Kummer E. Nat Commun 14 7991 (2023)
  2. Different Evolutionary Trends of Galloanseres: Mitogenomics Analysis. Zhou S, Wang X, Wang L, Gao X, Lyu T, Xia T, Shi L, Dong Y, Mei X, Zhang Z, Zhang H. Animals (Basel) 14 1437 (2024)
  3. NRhFluors: Quantitative Revealing the Interaction between Protein Homeostasis and Mitochondria Dysfunction via Fluorescence Lifetime Imaging. Huang Y, Chang M, Gao X, Fang J, Ding W, Liu J, Shen B, Zhang X. ACS Cent Sci 10 842-851 (2024)
  4. Small RNAs from mitochondrial genome recombination sites are incorporated into T. gondii mitoribosomes. Tetzlaff S, Hillebrand A, Drakoulis N, Gluhic Z, Maschmann S, Lyko P, Wicke S, Schmitz-Linneweber C. Elife 13 e95407 (2024)