ArticleProceedings of the National Academy of Sciences of the United States of America2016
Human mitochondrial ribosomes can switch their structural RNA composition.
Article in Proceedings of the National Academy of Sciences of the United States of America, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 51 papers.
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51 citing papers in PubMed, 79 citations in OpenAlex.
- The pseudouridine synthase PUSL1 modifies U39 of mitochondrial tRNAs and is linked to human neurological phenotypes.Nucleic acids research · 2026Article
- Expanding theJIMD reports · 2026Article
- Pseudouridine synthase PUS1 and initiation factor mtIF2 are human mitoribosomal small subunit assembly factors.Nature communications · 2026Article
- Structural basis of TACO1-mediated efficient mitochondrial translation.Nature communications · 2026Article
- Structural Basis of TACO1-Mediated Efficient Mitochondrial Translation.bioRxiv : the preprint server for biology · 2025Article
- Identification of a novel minor-groove DNA binder that represses mitochondrial gene expression and induces apoptosis in highly aggressive leiomyosarcoma cells.Cell death discovery · 2025Article
- Further delineation of defects in MRPS2 causing human OXPHOS deficiency and early developmental abnormalities in zebrafish.European journal of human genetics : EJHG · 2025Article
- The mitochondrial methylation potential gates mitoribosome assembly.Nature communications · 2025Article
- WBSCR16 is essential for mitochondrial 16S rRNA processing in mammals.Nucleic acids research · 2025Article
- DORQ-seq: high-throughput quantification of femtomol tRNA pools by combination of cDNA hybridization and Deep sequencing.Nucleic acids research · 2024Article
- Mitochondrial ribosome biogenesis and redox sensing.FEBS open bio · 2024Review
- Central dogma rates in human mitochondria.Human molecular genetics · 2024Review
- Illuminating mitochondrial translation through mouse models.Human molecular genetics · 2024Review
- CLPP-Null Eukaryotes with Excess Heme Biosynthesis Show Reduced L-arginine Levels, Probably via CLPX-Mediated OAT Activation.Biomolecules · 2024Article
- Molecular pathways in mitochondrial disorders due to a defective mitochondrial protein synthesis.Frontiers in cell and developmental biology · 2024Review
- Translation Fidelity and Respiration Deficits in CLPP-Deficient Tissues: Mechanistic Insights from Mitochondrial Complexome Profiling.International journal of molecular sciences · 2023Article
- Molecular Investigation of Mitochondrial RNA19 Role in the Pathogenesis of MELAS Disease.Life (Basel, Switzerland) · 2023Article
- Mitoribosome Biogenesis.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Evolution: Mitochondrial Ribosomes Across Species.Methods in molecular biology (Clifton, N.J.) · 2023Article
- Organization and expression of the mammalian mitochondrial genome.Nature reviews. Genetics · 2022Review
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7 authors at 5 institutions in 1 country.
Funding
Abstract
The recent developments in cryo-EM have revolutionized our access to previously refractory structures. In particular, such studies of mammalian mitoribosomes have confirmed the absence of any 5S rRNA species and revealed the unexpected presence of a mitochondrially encoded tRNA (mt-tRNA) that usurps this position. Although the cryo-EM structures resolved the conundrum of whether mammalian mitoribosomes contain a 5S rRNA, they introduced a new dilemma: Why do human and porcine mitoribosomes integrate contrasting mt-tRNAs? Human mitoribosomes have been shown to integrate mt-tRNA
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