ArticleNucleic acids research2018
Human GTPBP10 is required for mitoribosome maturation.
Article in Nucleic acids research, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 41 papers.
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Who cites it
41 citing papers in PubMed, 55 citations in OpenAlex.
- RMND1 dysfunction causes OXPHOS deficiency and alters mitoribosomal protein homeostasis in vivo.EMBO molecular medicine · 2026Article
- Peroxisomal Import Stress Drives Cellular Senescence through SCAF1-dependent Suppression of Mitoribosome Biogenesis.bioRxiv : the preprint server for biology · 2026Article
- Dysregulated circRNA-miRNA-mRNA networks reveal stage-specific mRNA expression changes in Parkinson's disease.Molecular brain · 2025Article
- WBSCR16 is essential for mitochondrial 16S rRNA processing in mammals.Nucleic acids research · 2025Article
- Bi-allelic variants in DAP3 result in reduced assembly of the mitoribosomal small subunit with altered apoptosis and a Perrault-syndrome-spectrum phenotype.American journal of human genetics · 2025Article
- Mitochondrial ribosome biogenesis and redox sensing.FEBS open bio · 2024Review
- Biallelic variants inmedRxiv : the preprint server for health sciences · 2024Article
- GTPBP8 plays a role in mitoribosome formation in human mitochondria.Nature communications · 2024Article
- Molecular pathways in mitochondrial disorders due to a defective mitochondrial protein synthesis.Frontiers in cell and developmental biology · 2024Review
- Structural insights into the role of GTPBP10 in the RNA maturation of the mitoribosome.Nature communications · 2023Article
- GTPBP8 is required for mitoribosomal biogenesis and mitochondrial translation.Cellular and molecular life sciences : CMLS · 2023Article
- OGT controls mammalian cell viability by regulating the proteasome/mTOR/ mitochondrial axis.Proceedings of the National Academy of Sciences of the United States of America · 2023Article
- Metabolic Labeling of Mitochondrial Translation Products in Whole Cells and Isolated Organelles.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Mitoribosome Biogenesis.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Systematic Analysis of Assembly Intermediates in Yeast to Decipher the Mitoribosome Assembly Pathway.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Article
- Functional analyses of mitoribosome 54S subunit devoid of mitochondria-specific protein sequences.Yeast (Chichester, England) · 2022Article
- MicroRNA editing patterns in Huntington's disease.Scientific reports · 2022Article
- A late-stage assembly checkpoint of the human mitochondrial ribosome large subunit.Nature communications · 2022Article
- Mechanisms of ribosome recycling in bacteria and mitochondria: a structural perspective.RNA biology · 2022Review
Corrections and comments
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Authors and funding
4 authors at 1 institution in 1 country.
Funding
Abstract
Most steps on the biogenesis of the mitochondrial ribosome (mitoribosome) occur near the mitochondrial DNA nucleoid, in RNA granules, which contain dedicated RNA metabolism and mitoribosome assembly factors. Here, analysis of the RNA granule proteome identified the presence of a set of small GTPases that belong to conserved families of ribosome assembly factors. We show that GTPBP10, a member of the conserved Obg family of P-loop small G proteins, is a mitochondrial protein and have used gene-editing technologies to create a HEK293T cell line KO for GTPBP10. The absence of GTPBP10 leads to attenuated mtLSU and mtSSU levels and the virtual absence of the 55S monosome, which entirely prevents mitochondrial protein synthesis. We show that a fraction of GTPBP10 cosediments with the large mitoribosome subunit and the monosome. GTPBP10 physically interacts with the 16S rRNA, but not with the 12S rRNA, and crosslinks with several mtLSU proteins. Additionally, GTPBP10 is indirectly required for efficient processing of the 12S-16S rRNA precursor transcript, which could explain the mtSSU accumulation defect. We propose that GTPBP10 primarily ensures proper mtLSU maturation and ultimately serves to coordinate mtSSU and mtLSU accumulation then providing a quality control check-point function during mtLSU assembly that minimizes premature subunit joining.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.