8QU5

mt-LSU assembly intermediate in GTPBP8 knock-out cells, state 2

Summary for 8QU5

• Entry DOI: 10.2210/pdb8qu5/pdb
• EMDB information: 18461 3843
• Descriptor: 39S ribosomal protein L32, mitochondrial, 39S ribosomal protein L13, mitochondrial, 39S ribosomal protein L14, mitochondrial, ... (58 entities in total)
• Functional Keywords: mitochondria, assembly, gtpbp8, ribosome
• Biological source: Homo sapiens (human); More
• Total number of polymer chains: 55
• Total formula weight: 1784436.59

Authors

Valentin Gese, G.,Cipullo, M.,Rorbach, J.,Hallberg, B.M. (deposition date: 2023-10-13, release date: 2024-06-26, Last modification date: 2024-07-17)

Primary citation

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

Experimental method

ELECTRON MICROSCOPY (2.42 Å)