ArticleNucleic acids research2018
MTG1 couples mitoribosome large subunit assembly with intersubunit bridge formation.
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 35 papers.
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Who cites it
35 citing papers in PubMed, 54 citations in OpenAlex.
- A five-gene mitochondria-associated prognostic signature for bladder cancer.International journal of clinical and experimental pathology · 2026Article
- The late stages of yeast mitoribosome large subunit biogenesis.Biochimica et biophysica acta. Molecular cell research · 2025Article
- Mapping in silico genetic networks of the KMT2D tumour suppressor gene to uncover novel functional associations and cancer cell vulnerabilities.Genome medicine · 2024Article
- Mitochondrial ribosome biogenesis and redox sensing.FEBS open bio · 2024Review
- The human mitochondrial translation factor TACO1 alleviates mitoribosome stalling at polyproline stretches.Nucleic acids research · 2024Article
- The human mitochondrial mRNA structurome reveals mechanisms of gene expression.Science (New York, N.Y.) · 2024Article
- Supernumerary proteins of the human mitochondrial ribosomal small subunit are integral for assembly and translation.iScience · 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
- Structural analysis of mitochondrial rRNA gene variants identified in patients with deafness.Frontiers in physiology · 2023Article
- Mitoribosome Biogenesis.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Mechanisms of ribosome recycling in bacteria and mitochondria: a structural perspective.RNA biology · 2022Review
- MRPS31 loss is a key driver of mitochondrial deregulation and hepatocellular carcinoma aggressiveness.Cell death & disease · 2021Article
- Article
- Structural basis of GTPase-mediated mitochondrial ribosome biogenesis and recycling.Nature communications · 2021Article
- Stepwise maturation of the peptidyl transferase region of human mitoribosomes.Nature communications · 2021Article
- Structural basis for late maturation steps of the human mitoribosomal large subunit.Nature communications · 2021Article
- YbeY is required for ribosome small subunit assembly and tRNA processing in human mitochondria.Nucleic acids research · 2021Article
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Authors and funding
2 authors at 1 institution in 1 country.
Funding
Abstract
Mammalian mitochondrial ribosomes (mitoribosomes) synthesize 13 proteins, essential components of the oxidative phosphorylation system. They are linked to mitochondrial disorders, often involving cardiomyopathy. Mitoribosome biogenesis is assisted by multiple cofactors whose specific functions remain largely uncharacterized. Here, we examined the role of human MTG1, a conserved ribosome assembly guanosine triphosphatase. MTG1-silencing in human cardiomyocytes and developing zebrafish revealed early cardiovascular lesions. A combination of gene-editing and biochemical approaches using HEK293T cells demonstrated that MTG1 binds to the large subunit (mtLSU) 16S ribosomal RNA to facilitate incorporation of late-assembly proteins. Furthermore, MTG1 interacts with mtLSU uL19 protein and mtSSU mS27, a putative guanosine triphosphate-exchange factor (GEF), to enable MTG1 release and the formation of the mB6 intersubunit bridge. In this way, MTG1 establishes a quality control checkpoint in mitoribosome assembly. In conclusion, MTG1 controls mitochondrial translation by coupling mtLSU assembly with intersubunit bridge formation using the intrinsic GEF activity acquired by the mtSSU through mS27, a unique occurrence in translational systems.
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