9EY2

Human mitochondrial RNase Z with tRNA-His-CCA

Summary for 9EY2

• Entry DOI: 10.2210/pdb9ey2/pdb
• EMDB information: 50052 51230
• Descriptor: 3-hydroxyacyl-CoA dehydrogenase type-2, tRNA methyltransferase 10 homolog C, mt-tRNA-His-CCA, ... (7 entities in total)
• Functional Keywords: rnase z, elac2, mitochondria, trna, rna binding protein
• Biological source: Homo sapiens (human); More
• Total number of polymer chains: 7
• Total formula weight: 262664.61

Authors

Valentin Gese, G.,Hallberg, B.M. (deposition date: 2024-04-09, release date: 2024-11-27, Last modification date: 2024-12-25)

Primary citation

Valentin Gese, G.,Hallberg, B.M.
Structural basis of 3'-tRNA maturation by the human mitochondrial RNase Z complex.
Embo J., 43:6573-6590, 2024

PubMed 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 tRNA, 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.

PubMed: 39516281
DOI: 10.1038/s44318-024-00297-w

Experimental method

ELECTRON MICROSCOPY (2.96 Å)