| Structural highlights
8oit is a 15 chain structure with sequence from Homo sapiens. Full crystallographic information is available from OCA. For a guided tour on the structure components use FirstGlance.
| | Method: | Electron Microscopy, Resolution 2.9Å |
| Ligands: | , , , , , , , , , , , , , , |
| Resources: | FirstGlance, OCA, PDBe, RCSB, PDBsum, ProSAT |
Disease
RM12_HUMAN The disease may be caused by variants affecting the gene represented in this entry.
Function
RM12_HUMAN As a component of the mitochondrial large ribosomal subunit, it plays a role in mitochondrial translation (PubMed:23603806). Associates with mitochondrial RNA polymerase to activate transcription.[1] [2]
Publication Abstract from PubMed
The genetic code that specifies the identity of amino acids incorporated into proteins during protein synthesis is almost universally conserved. Mitochondrial genomes feature deviations from the standard genetic code, including the reassignment of two arginine codons to stop codons. The protein required for translation termination at these noncanonical stop codons to release the newly synthesized polypeptides is not currently known. In this study, we used gene editing and ribosomal profiling in combination with cryo-electron microscopy to establish that mitochondrial release factor 1 (mtRF1) detects noncanonical stop codons in human mitochondria by a previously unknown mechanism of codon recognition. We discovered that binding of mtRF1 to the decoding center of the ribosome stabilizes a highly unusual conformation in the messenger RNA in which the ribosomal RNA participates in specific recognition of the noncanonical stop codons.
Molecular basis of translation termination at noncanonical stop codons in human mitochondria.,Saurer M, Leibundgut M, Nadimpalli HP, Scaiola A, Schonhut T, Lee RG, Siira SJ, Rackham O, Dreos R, Lenarcic T, Kummer E, Gatfield D, Filipovska A, Ban N Science. 2023 May 5;380(6644):531-536. doi: 10.1126/science.adf9890. Epub 2023 , May 4. PMID:37141370[3]
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.
References
- ↑ Surovtseva YV, Shutt TE, Cotney J, Cimen H, Chen SY, Koc EC, Shadel GS. Mitochondrial ribosomal protein L12 selectively associates with human mitochondrial RNA polymerase to activate transcription. Proc Natl Acad Sci U S A. 2011 Nov 1;108(44):17921-6. PMID:22003127 doi:10.1073/pnas.1108852108
- ↑ Serre V, Rozanska A, Beinat M, Chretien D, Boddaert N, Munnich A, Rötig A, Chrzanowska-Lightowlers ZM. Mutations in mitochondrial ribosomal protein MRPL12 leads to growth retardation, neurological deterioration and mitochondrial translation deficiency. Biochim Biophys Acta. 2013 Aug;1832(8):1304-12. PMID:23603806 doi:10.1016/j.bbadis.2013.04.014
- ↑ Saurer M, Leibundgut M, Nadimpalli HP, Scaiola A, Schönhut T, Lee RG, Siira SJ, Rackham O, Dreos R, Lenarčič T, Kummer E, Gatfield D, Filipovska A, Ban N. Molecular basis of translation termination at noncanonical stop codons in human mitochondria. Science. 2023 May 5;380(6644):531-536. PMID:37141370 doi:10.1126/science.adf9890
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