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-Structure paper
| タイトル | Structural basis of 3'-tRNA maturation by the human mitochondrial RNase Z complex. |
|---|---|
| ジャーナル・号・ページ | EMBO J, Vol. 43, Issue 24, Page 6573-6590, Year 2024 |
| 掲載日 | 2024年11月8日 |
著者 | Genís Valentín Gesé / B Martin Hällberg / ![]() |
| PubMed 要旨 | Maturation of human mitochondrial tRNA is essential for cellular energy production, yet the underlying mechanisms remain only partially understood. Here, we present several cryo-EM structures of the ...Maturation of human mitochondrial tRNA is essential for cellular energy production, yet the underlying mechanisms remain only partially understood. Here, we present several cryo-EM structures of the mitochondrial RNase Z complex (ELAC2/SDR5C1/TRMT10C) bound to different maturation states of mitochondrial tRNA, showing the molecular basis for tRNA-substrate selection and catalysis. Our structural insights provide a molecular rationale for the 5'-to-3' tRNA processing order in mitochondria, the 3'-CCA antideterminant effect, and the basis for sequence-independent recognition of mitochondrial tRNA substrates. Furthermore, our study links mutations in ELAC2 to clinically relevant mitochondrial diseases, offering a deeper understanding of the molecular defects contributing to these conditions. |
リンク | EMBO J / PubMed:39516281 / PubMed Central |
| 手法 | EM (単粒子) |
| 解像度 | 1.9 - 3.34 Å |
| 構造データ | ![]() EMDB-19954: Human mitochondrial RNase Z with tRNA-His, ELAC2 focused map ![]() EMDB-19955: Human mitochondrial RNase Z with tRNA-His, consensus map ![]() EMDB-19956: Human mitochondrial RNase Z with tRNA-His precursor, consensus map ![]() EMDB-19957: Human mitochondrial RNase Z with tRNA-His precursor, ELAC2 focused map ![]() EMDB-19958: Human mitochondrial RNase Z with tRNA-His-CCA, consensus map ![]() EMDB-19959: Human mitochondrial RNase Z with tRNA-His-CCA, ELAC2 focused map EMDB-50050, PDB-9ey0: EMDB-50051, PDB-9ey1: EMDB-50052, PDB-9ey2: EMDB-51230, PDB-9gch: |
| 化合物 | ![]() ChemComp-ZN: ![]() ChemComp-GTP: ![]() ChemComp-SAM: ![]() ChemComp-SAH: ![]() ChemComp-MG: ![]() ChemComp-HOH: |
| 由来 |
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キーワード | RNA BINDING PROTEIN / RNAseZ / ELAC2 / mitochondria / tRNA / RNase Z |
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