9EY1

Human mitochondrial RNase Z with tRNA-His precursor

9EY1 の概要

• エントリーDOI: 10.2210/pdb9ey1/pdb
• EMDBエントリー: 50051
• 分子名称: Zinc phosphodiesterase ELAC protein 2, 3-hydroxyacyl-CoA dehydrogenase type-2, tRNA-His-tRNA-Ser, ... (7 entities in total)
• 機能のキーワード: rnasez, elac2, mitochondria, trna, rna binding protein
• 由来する生物種: Homo sapiens (human); 詳細
• タンパク質・核酸の鎖数: 7
• 化学式量合計: 280427.74

構造登録者

Valentin Gese, G.,Hallberg, B.M. (登録日: 2024-04-09, 公開日: 2024-11-13, 最終更新日: 2025-12-10)

主引用文献

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

実験手法

ELECTRON MICROSCOPY (2.9 Å)