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Structural basis of 3'-tRNA maturation by the human mitochondrial RNase Z complex.

Valentín Gesé G, Hällberg BM
EMBO J (2024)
Related entries: 9ey0, 9ey2, 9gch

Cited: 1 times
EuropePMC logo PMID: 39516281

Abstract

Maturation of human mitochondrial tRNA is essential for cellular energy production, yet the underlying mechanisms remain only partially understood. Here, we present several cryo-EM structures of the mitochondrial RNase Z complex (ELAC2/SDR5C1/TRMT10C) bound to different maturation states of mitochondrial tRNAHis, showing the molecular basis for tRNA-substrate selection and catalysis. Our structural insights provide a molecular rationale for the 5'-to-3' tRNA processing order in mitochondria, the 3'-CCA antideterminant effect, and the basis for sequence-independent recognition of mitochondrial tRNA substrates. Furthermore, our study links mutations in ELAC2 to clinically relevant mitochondrial diseases, offering a deeper understanding of the molecular defects contributing to these conditions.

Articles citing this publication (1)

  1. Structural insights into human ELAC2 as a tRNA 3' processing enzyme. Xue C, Tian J, Chen Y, Liu Z. Nucleic Acids Res 52 13434-13446 (2024)


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