Human mtRF1 terminates COX1 translation and its ablation induces mitochondrial ribosome-associated quality control

Franziska Nadler, Elena Lavdovskaia, Angelique Krempler, Luis Daniel Cruz-Zaragoza, Sven Dennerlein and Ricarda Richter-Dennerlein ()
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Franziska Nadler: University Medical Center Goettingen
Elena Lavdovskaia: University Medical Center Goettingen
Angelique Krempler: University Medical Center Goettingen
Luis Daniel Cruz-Zaragoza: University Medical Center Goettingen
Sven Dennerlein: University Medical Center Goettingen
Ricarda Richter-Dennerlein: University Medical Center Goettingen

Nature Communications, 2022, vol. 13, issue 1, 1-10

Abstract: Abstract Translation termination requires release factors that read a STOP codon in the decoding center and subsequently facilitate the hydrolysis of the nascent peptide chain from the peptidyl tRNA within the ribosome. In human mitochondria eleven open reading frames terminate in the standard UAA or UAG STOP codon, which can be recognized by mtRF1a, the proposed major mitochondrial release factor. However, two transcripts encoding for COX1 and ND6 terminate in the non-conventional AGA or AGG codon, respectively. How translation termination is achieved in these two cases is not known. We address this long-standing open question by showing that the non-canonical release factor mtRF1 is a specialized release factor that triggers COX1 translation termination, while mtRF1a terminates the majority of other mitochondrial translation events including the non-canonical ND6. Loss of mtRF1 leads to isolated COX deficiency and activates the mitochondrial ribosome-associated quality control accompanied by the degradation of COX1 mRNA to prevent an overload of the ribosome rescue system. Taken together, these results establish the role of mtRF1 in mitochondrial translation, which had been a mystery for decades, and lead to a comprehensive picture of translation termination in human mitochondria.

Date: 2022
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DOI: 10.1038/s41467-022-34088-w

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