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-Structure paper
Title | Coupling of ribosome biogenesis and translation initiation in human mitochondria. |
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Journal, issue, pages | Nat Commun, Vol. 16, Issue 1, Page 3641, Year 2025 |
Publish date | Apr 17, 2025 |
![]() | Marleen Heinrichs / Anna Franziska Finke / Shintaro Aibara / Angelique Krempler / Angela Boshnakovska / Peter Rehling / Hauke S Hillen / Ricarda Richter-Dennerlein / ![]() |
PubMed Abstract | Biogenesis of mitoribosomes requires dedicated chaperones, RNA-modifying enzymes, and GTPases, and defects in mitoribosome assembly lead to severe mitochondriopathies in humans. Here, we characterize ...Biogenesis of mitoribosomes requires dedicated chaperones, RNA-modifying enzymes, and GTPases, and defects in mitoribosome assembly lead to severe mitochondriopathies in humans. Here, we characterize late-step assembly states of the small mitoribosomal subunit (mtSSU) by combining genetic perturbation and mutagenesis analysis with biochemical and structural approaches. Isolation of native mtSSU biogenesis intermediates via a FLAG-tagged variant of the GTPase MTG3 reveals three distinct assembly states, which show how factors cooperate to mature the 12S rRNA. In addition, we observe four distinct primed initiation mtSSU states with an incompletely matured rRNA, suggesting that biogenesis and translation initiation are not mutually exclusive processes but can occur simultaneously. Together, these results provide insights into mtSSU biogenesis and suggest a functional coupling between ribosome biogenesis and translation initiation in human mitochondria. |
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Methods | EM (single particle) |
Resolution | 3.0 - 3.8 Å |
Structure data | ![]() EMDB-51039: Translation-initiation state of human mitochondrial ribosome small subunit (State G) ![]() EMDB-51042: Translation-initiation state of human mitochondrial ribosome small subunit (State G) ![]() EMDB-51043: Translation-initiation state of human mitochondrial ribosome small subunit (State E) ![]() EMDB-51045: Translation-initiation state of human mitochondrial ribosome small subunit (State E) ![]() EMDB-51046: Translation-initiation state of human mitochondrial ribosome small subunit (State D) ![]() EMDB-51047: Translation-initiation state of human mitochondrial ribosome small subunit (State D) ![]() EMDB-51048: Translation-initiation state of human mitochondrial ribosome small subunit (State F) ![]() EMDB-51049: Translation-initiation state of human mitochondrial ribosome small subunit (State F) ![]() EMDB-51050: Translation-initiation state of human mitochondrial ribosome small subunit (State F) ![]() EMDB-51051: Assembly intermediate of human mitochondrial ribosome small subunit (State C) ![]() EMDB-51052: Assembly intermediate of human mitochondrial ribosome small subunit (State B) ![]() EMDB-51053: Local B-factor-filtered map of an assembly intermediate of human mitochondrial ribosome small subunit (State A) ![]() EMDB-51054: Translation-initiation state of human mitochondrial ribosome small subunit (State F) ![]() EMDB-51055: Translation-initiation state of human mitochondrial ribosome small subunit (State F) ![]() EMDB-51056: Assembly intermediate of human mitochondrial ribosome small subunit (State C) ![]() EMDB-51057: Assembly intermediate of human mitochondrial ribosome small subunit (State C) ![]() EMDB-51058: Assembly intermediate of human mitochondrial ribosome small subunit (State C) ![]() EMDB-51059: Assembly intermediate of human mitochondrial ribosome small subunit (State C) ![]() EMDB-51060: Assembly intermediate of human mitochondrial ribosome small subunit (State B) ![]() EMDB-51061: Assembly intermediate of human mitochondrial ribosome small subunit (State B) ![]() EMDB-51062: Assembly intermediate of human mitochondrial ribosome small subunit (State B) ![]() EMDB-51063: Assembly intermediate of human mitochondrial ribosome small subunit (State B) ![]() EMDB-51064: Head-focused refinement of an assembly intermediate of human mitochondrial ribosome small subunit (State A) ![]() EMDB-51065: Body-focused refinement of an assembly intermediate of human mitochondrial ribosome small subunit (State A) ![]() EMDB-51066: Consensus refinement of an assembly intermediate of human mitochondrial ribosome small subunit (State A) EMDB-51083, PDB-9g5b: EMDB-51084, PDB-9g5c: EMDB-51085, PDB-9g5d: EMDB-51086, PDB-9g5e: ![]() EMDB-52062: Translation-initiation state of human mitochondrial ribosome small subunit (State D) ![]() EMDB-52063: Translation-initiation state of human mitochondrial ribosome small subunit (State D) ![]() EMDB-52064: Translation-initiation state of human mitochondrial ribosome small subunit (State E) ![]() EMDB-52065: Translation-initiation state of human mitochondrial ribosome small subunit (State E) ![]() EMDB-52066: Translation-initiation state of human mitochondrial ribosome small subunit (State G) ![]() EMDB-52067: Translation-initiation state of human mitochondrial ribosome small subunit (State G) ![]() EMDB-52068: Translation-initiation state of human mitochondrial ribosome small subunit (State G) EMDB-52117, PDB-9hfm: EMDB-52118, PDB-9hfn: EMDB-52119, PDB-9hfo: |
Chemicals | ![]() ChemComp-MG: ![]() ChemComp-ZN: ![]() ChemComp-FES: ![]() ChemComp-ATP: ![]() ChemComp-GDP: ![]() ChemComp-K: ![]() ChemComp-SAH: ![]() ChemComp-NAD: ![]() ChemComp-GTP: |
Source |
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![]() | RIBOSOME / mitochondrial ribosomal small subunit / assembly intermediate / MTG3 / single-particle cryo-EM / immature h44 / translation-initiation intermediate |