{"title":"Advances in Human Pre-tRNA Maturation: TRMT10C and ELAC2 in Focus","authors":"Juhi Sikarwar, Vincent Meynier, Carine Tisné","doi":"10.1016/j.jmb.2025.168989","DOIUrl":null,"url":null,"abstract":"<div><div>Mitochondrial pre-tRNA maturation is a multi-step process involving the removal of the 5′-leader by PRORP, 3′-trailer processing by ELAC2, 3′-CCA addition by TRNT1, and the incorporation of post-transcriptional modifications. In metazoans, the low structural stability of mitochondrial pre-tRNAs adds significant complexity to these steps, and defects in their maturation have been implicated in various human mitochondrial disorders. In this case, the tRNA methyltransferase complex TRMT10C/SDR5C1 compensates for the pre-tRNA structural alteration to present the pre-tRNA to maturation enzymes. Cryo-electron microscopy structures of human mitochondrial pre-tRNA maturation complexes have provided critical insights into these essential processes. Here we review the current understanding of tRNA maturation within human mitochondria and explore its implications for nuclear pre-tRNA maturation.</div></div>","PeriodicalId":369,"journal":{"name":"Journal of Molecular Biology","volume":"437 16","pages":"Article 168989"},"PeriodicalIF":4.5000,"publicationDate":"2025-08-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"Journal of Molecular Biology","FirstCategoryId":"99","ListUrlMain":"https://www.sciencedirect.com/science/article/pii/S0022283625000555","RegionNum":2,"RegionCategory":"生物学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"2025/2/10 0:00:00","PubModel":"Epub","JCR":"Q1","JCRName":"BIOCHEMISTRY & MOLECULAR BIOLOGY","Score":null,"Total":0}
引用次数: 0
Abstract
Mitochondrial pre-tRNA maturation is a multi-step process involving the removal of the 5′-leader by PRORP, 3′-trailer processing by ELAC2, 3′-CCA addition by TRNT1, and the incorporation of post-transcriptional modifications. In metazoans, the low structural stability of mitochondrial pre-tRNAs adds significant complexity to these steps, and defects in their maturation have been implicated in various human mitochondrial disorders. In this case, the tRNA methyltransferase complex TRMT10C/SDR5C1 compensates for the pre-tRNA structural alteration to present the pre-tRNA to maturation enzymes. Cryo-electron microscopy structures of human mitochondrial pre-tRNA maturation complexes have provided critical insights into these essential processes. Here we review the current understanding of tRNA maturation within human mitochondria and explore its implications for nuclear pre-tRNA maturation.
期刊介绍：
Journal of Molecular Biology (JMB) provides high quality, comprehensive and broad coverage in all areas of molecular biology. The journal publishes original scientific research papers that provide mechanistic and functional insights and report a significant advance to the field. The journal encourages the submission of multidisciplinary studies that use complementary experimental and computational approaches to address challenging biological questions.
Research areas include but are not limited to: Biomolecular interactions, signaling networks, systems biology; Cell cycle, cell growth, cell differentiation; Cell death, autophagy; Cell signaling and regulation; Chemical biology; Computational biology, in combination with experimental studies; DNA replication, repair, and recombination; Development, regenerative biology, mechanistic and functional studies of stem cells; Epigenetics, chromatin structure and function; Gene expression; Membrane processes, cell surface proteins and cell-cell interactions; Methodological advances, both experimental and theoretical, including databases; Microbiology, virology, and interactions with the host or environment; Microbiota mechanistic and functional studies; Nuclear organization; Post-translational modifications, proteomics; Processing and function of biologically important macromolecules and complexes; Molecular basis of disease; RNA processing, structure and functions of non-coding RNAs, transcription; Sorting, spatiotemporal organization, trafficking; Structural biology; Synthetic biology; Translation, protein folding, chaperones, protein degradation and quality control.
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