Work

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

Year: 2024

Type: article

Abstract: Human mitochondrial tRNA maturation 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 tRNA His , 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, the study links mutations in ELAC2 to clinically relevant mitochondrial diseases, offering a deeper understanding of the molecular defects contributing to these conditions.

Source: The EMBO Journal

DOI: 10.1038/s44318-024-00297-w

Authors: Genís Valentín Gesé, B Martin Hällberg

Institutions: Karolinska Institutet, Centre for Structural Systems Biology

Language: English

Topic: RNA and protein synthesis mechanisms

Subfield: Molecular Biology

Field: Biochemistry, Genetics and Molecular Biology

Domain: Life Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $6,890