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Updated: Jun 14, 2025

An In Vitro Assay to Detect tRNA-Isopentenyl Transferase Activity
Published on: October 8, 2018
Advances in Human Pre-tRNA Maturation: TRMT10C and ELAC2 in Focus
Juhi Sikarwar1, Vincent Meynier1, Carine Tisné1
1Université Paris Cité, CNRS, Institut de Biologie Physico-Chimique (IBPC), Microbial Gene Expression Unit, 75005 Paris, France.
None:
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.
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