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-Structure paper
タイトル | GTPBP8 plays a role in mitoribosome formation in human mitochondria. |
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ジャーナル・号・ページ | Nat Commun, Vol. 15, Issue 1, Page 5664, Year 2024 |
掲載日 | 2024年7月5日 |
著者 | Miriam Cipullo / Genís Valentín Gesé / Shreekara Gopalakrishna / Annika Krueger / Vivian Lobo / Maria A Pirozhkova / James Marks / Petra Páleníková / Dmitrii Shiriaev / Yong Liu / Jelena Misic / Yu Cai / Minh Duc Nguyen / Abubakar Abdelbagi / Xinping Li / Michal Minczuk / Markus Hafner / Daniel Benhalevy / Aishe A Sarshad / Ilian Atanassov / B Martin Hällberg / Joanna Rorbach / |
PubMed 要旨 | Mitochondrial gene expression relies on mitoribosomes to translate mitochondrial mRNAs. The biogenesis of mitoribosomes is an intricate process involving multiple assembly factors. Among these ...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. |
リンク | Nat Commun / PubMed:38969660 / PubMed Central |
手法 | EM (単粒子) |
解像度 | 2.42 - 6.75 Å |
構造データ | EMDB-18438, PDB-8qrk: EMDB-18439, PDB-8qrl: EMDB-18440, PDB-8qrm: EMDB-18443: mt-SSU assembly intermediate in GTPBP8 knock-out cells, state 4 EMDB-18460, PDB-8qu1: EMDB-18461, PDB-8qu5: |
化合物 | ChemComp-MG: ChemComp-NAD: ChemComp-SPM: ChemComp-SRY: ChemComp-K: ChemComp-ZN: ChemComp-FES: ChemComp-ATP: ChemComp-GNP: ChemComp-GDP: ChemComp-SAH: ChemComp-HOH: ChemComp-FME: ChemComp-GTP: ChemComp-PNS: |
由来 |
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キーワード | RIBOSOME / Mitochondria / Assembly / GTPBP8 |