9EY0
Human mitochondrial RNase Z with tRNA-His
Summary for 9EY0
| Entry DOI | 10.2210/pdb9ey0/pdb |
| EMDB information | 50050 |
| Descriptor | 3-hydroxyacyl-CoA dehydrogenase type-2, Zinc phosphodiesterase ELAC protein 2, mt-tRNA-His, ... (7 entities in total) |
| Functional Keywords | rnasez, elac2, mitochondria, trna, rna binding protein |
| Biological source | Homo sapiens (human) More |
| Total number of polymer chains | 7 |
| Total formula weight | 262664.61 |
| Authors | Valentin Gese, G.,Hallberg, B.M. (deposition date: 2024-04-09, release date: 2024-11-13, Last modification date: 2024-12-25) |
| Primary citation | Valentin Gese, G.,Hallberg, B.M. Structural basis of 3'-tRNA maturation by the human mitochondrial RNase Z complex. Embo J., 43:6573-6590, 2024 Cited by PubMed Abstract: 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. PubMed: 39516281DOI: 10.1038/s44318-024-00297-w PDB entries with the same primary citation |
| Experimental method | ELECTRON MICROSCOPY (2.78 Å) |
Structure validation
Download full validation report
