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Mitochondrial tRNA biogenesis, modifications and disease relevance
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DOI：10.1038/s41580-026-00999-5.png)
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Abstract
En 中文
The biogenesis, modifications and function of mitochondrial transfer RNAs (mt-tRNAs) reflect the symbiotic relationship and coordinated evolution between the domesticated organelle and the outer cell. Through evolution, mt-tRNA structures have been severely degenerated, and mt-tRNA-associated proteomes have acquired additional domains and interfaces, leveraging post-transcriptional modifications to maintain functional affinity and specificity. Considerable progress has been made in the past decade in elucidating mt-tRNA structure, biogenesis, modifications and functions. In this Review, we outline how mt-tRNAs are excised from polycistronic transcripts and mature through coordinated actions of mitochondrial processing enzymes. We then examine how mitochondrial aminoacyl-tRNA synthetases and mitoribosomes have coevolved to recognize degenerated mt-tRNAs and support a streamlined genetic code. The roles of post-transcriptional modifications in mt-tRNA structure stabilization, mt-tRNA decoding and the coupling of metabolism to translation are also discussed. Moreover, we review mt-tRNA-associated pathologies and emerging therapeutic strategies, highlighting unifying principles that inform efforts to restore coherence of mitochondrial translation. Mitochondrial tRNA (mt-tRNA) biogenesis has evolved to support a degenerate mt-tRNA structure and a simplified genetic code. Liu and colleagues provide a fascinating overview of mt-tRNA biology, discuss how mt-tRNA-associated perturbations directly cause disease, and examine how emerging knowledge will lead to targeted therapeutics.
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