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-Structure paper
| タイトル | Pseudouridine synthase PUS1 and initiation factor mtIF2 are human mitoribosomal small subunit assembly factors. |
|---|---|
| ジャーナル・号・ページ | Nat Commun, Vol. 17, Year 2026 |
| 掲載日 | 2026年4月21日 |
著者 | Vivek Singh / Dmitrii Shiriaev / Lorina Bilalli / Anas Khawaja / Joanna Rorbach / ![]() |
| PubMed 要旨 | Assembly of the mitochondrial ribosome (mitoribosome) is a crucial step in mitochondrial gene expression. This process facilitates mitochondrial translation, which produces essential subunits of the ...Assembly of the mitochondrial ribosome (mitoribosome) is a crucial step in mitochondrial gene expression. This process facilitates mitochondrial translation, which produces essential subunits of the oxidative phosphorylation machinery-the cell's primary energy-producing machinery. Disruptions in mitoribosome assembly can lead to severe human diseases. Given its fundamental importance, detailed structural analysis of mitoribosome assembly pathways is essential for advancing our understanding of mitochondrial function in both health and disease. In this study, we characterize twelve distinct assembly states of the mitoribosomal small subunit (mtSSU) isolated from human cells. Our findings reveal the intricate details of the final maturation stages of the mtSSU platform, decoding center, and the 3'-end of 12S rRNA. This process is governed by coordinated actions of assembly factors that ensure precise, stepwise rRNA folding and the integration of mitoribosomal proteins into the developing subunit. Our approach identifies pseudouridine synthase PUS1 and initiation factor mtIF2 as assembly factors, expanding their known roles beyond mt-tRNA maturation and translation, respectively. In addition, the identified assembly intermediates provide insight into the modular nature of mtSSU biogenesis in mitochondria and further link late-stage assembly to the acquisition of translational competence. |
リンク | Nat Commun / PubMed:42342677 / PubMed Central |
| 手法 | EM (単粒子) |
| 解像度 | 3.0 - 4.6 Å |
| 構造データ | EMDB-51873, PDB-9h51: EMDB-51874, PDB-9h52: EMDB-51876, PDB-9h54: EMDB-51877, PDB-9h55: EMDB-54129, PDB-9ror: EMDB-54130, PDB-9rot: EMDB-54131, PDB-9rov: EMDB-54163, PDB-9rpf: ![]() EMDB-57663: human mitochondrial ribosome small subunit: Initiation complex with IF3 (State I2) ![]() EMDB-57667: Human mitochondrial small ribosomal subunit: Initiation complex with IF2 and IF3 (State I3) ![]() EMDB-57668: Human mitochondrial small ribosomal subunit: Initiation complex with IF2 and tRNA (state I4) ![]() EMDB-57669: Human mitochondrial small ribosomal subunit: Assembly intermediate with IF3 and RBFA-out (state I1) |
| 化合物 | ![]() ChemComp-NAD: ![]() ChemComp-SPM: ![]() ChemComp-MG: ![]() ChemComp-K: ![]() ChemComp-ZN: ![]() ChemComp-FES: ![]() ChemComp-SAH: ![]() ChemComp-ATP: ![]() ChemComp-GDP: ![]() ChemComp-HOH: ![]() ChemComp-SF4: |
| 由来 |
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キーワード | RIBOSOME / mitochondria / small subunit / assembly / NOA1 / mitochondrial gene expression / mitoribosome / mitoribosome assembly factors / cryoEM |
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homo sapiens (ヒト)
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