Human mitochondria require mtRF1 for translation termination at non-canonical stop codons

Annika Krüger, Cristina Remes, Dmitrii Igorevich Shiriaev, Yong Liu, Henrik Spåhr, Rolf Wibom, Ilian Atanassov, Minh Duc Nguyen, Barry S. Cooperman and Joanna Rorbach ()
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Annika Krüger: Karolinska Institutet, Biomedicum
Cristina Remes: University of Pennsylvania
Dmitrii Igorevich Shiriaev: Karolinska Institutet, Biomedicum
Yong Liu: Karolinska Institutet, Biomedicum
Henrik Spåhr: Karolinska Institutet, Biomedicum
Rolf Wibom: Karolinska Institutet
Ilian Atanassov: Max-Planck-Institute for Biology of Ageing
Minh Duc Nguyen: Karolinska Institutet, Biomedicum
Barry S. Cooperman: University of Pennsylvania
Joanna Rorbach: Karolinska Institutet, Biomedicum

Nature Communications, 2023, vol. 14, issue 1, 1-16

Abstract: Abstract The mitochondrial translation machinery highly diverged from its bacterial counterpart. This includes deviation from the universal genetic code, with AGA and AGG codons lacking cognate tRNAs in human mitochondria. The locations of these codons at the end of COX1 and ND6 open reading frames, respectively, suggest they might function as stop codons. However, while the canonical stop codons UAA and UAG are known to be recognized by mtRF1a, the release mechanism at AGA and AGG codons remains a debated issue. Here, we show that upon the loss of another member of the mitochondrial release factor family, mtRF1, mitoribosomes accumulate specifically at AGA and AGG codons. Stalling of mitoribosomes alters COX1 transcript and protein levels, but not ND6 synthesis. In addition, using an in vitro reconstituted mitochondrial translation system, we demonstrate the specific peptide release activity of mtRF1 at the AGA and AGG codons. Together, our results reveal the role of mtRF1 in translation termination at non-canonical stop codons in mitochondria.

Date: 2023
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DOI: 10.1038/s41467-022-35684-6

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