8QRM

mt-SSU assembly intermediate in GTPBP8 knock-out cells, state 3

8QRM の概要

• エントリーDOI: 10.2210/pdb8qrm/pdb
• EMDBエントリー: 18440
• 分子名称: 12S mitochondrial rRNA, 28S ribosomal protein S12, mitochondrial, 28S ribosomal protein S14, mitochondrial, ... (41 entities in total)
• 機能のキーワード: mitochondria, assembly, gtpbp8, ribosome
• 由来する生物種: Homo sapiens (human); 詳細
• タンパク質・核酸の鎖数: 32
• 化学式量合計: 1179545.62

構造登録者

Valentin Gese, G.,Cipullo, M.,Rorbach, J.,Hallberg, B.M. (登録日: 2023-10-09, 公開日: 2024-06-26, 最終更新日: 2024-07-17)

主引用文献

Cipullo, M.,Valentin Gese, G.,Gopalakrishna, S.,Krueger, A.,Lobo, V.,Pirozhkova, M.A.,Marks, J.,Palenikova, P.,Shiriaev, D.,Liu, Y.,Misic, J.,Cai, Y.,Nguyen, M.D.,Abdelbagi, A.,Li, X.,Minczuk, M.,Hafner, M.,Benhalevy, D.,Sarshad, A.A.,Atanassov, I.,Hallberg, B.M.,Rorbach, J.
GTPBP8 plays a role in mitoribosome formation in human mitochondria.
Nat Commun, 15:5664-5664, 2024

PubMed Abstract: Mitochondrial gene expression relies on mitoribosomes to translate mitochondrial mRNAs. The biogenesis of mitoribosomes is an intricate process involving multiple assembly factors. Among these factors, GTP-binding proteins (GTPBPs) play important roles. In bacterial systems, numerous GTPBPs are required for ribosome subunit maturation, with EngB being a GTPBP involved in the ribosomal large subunit assembly. In this study, we focus on exploring the function of GTPBP8, the human homolog of EngB. We find that ablation of GTPBP8 leads to the inhibition of mitochondrial translation, resulting in significant impairment of oxidative phosphorylation. Structural analysis of mitoribosomes from GTPBP8 knock-out cells shows the accumulation of mitoribosomal large subunit assembly intermediates that are incapable of forming functional monosomes. Furthermore, fPAR-CLIP analysis reveals that GTPBP8 is an RNA-binding protein that interacts specifically with the mitochondrial ribosome large subunit 16 S rRNA. Our study highlights the role of GTPBP8 as a component of the mitochondrial gene expression machinery involved in mitochondrial large subunit maturation.

PubMed: 38969660
DOI: 10.1038/s41467-024-50011-x

実験手法

ELECTRON MICROSCOPY (3.05 Å)