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| ==Structure of the human mitochondrial ribosome (class 1)== | | ==Structure of the human mitochondrial ribosome (class 1)== |
| <StructureSection load='3j9m' size='340' side='right' caption='[[3j9m]], [[Resolution|resolution]] 3.50Å' scene=''> | | <SX load='3j9m' size='340' side='right' viewer='molstar' caption='[[3j9m]], [[Resolution|resolution]] 3.50Å' scene=''> |
| == Structural highlights == | | == Structural highlights == |
| <table><tr><td colspan='2'>[[3j9m]] is a 84 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3J9M OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3J9M FirstGlance]. <br> | | <table><tr><td colspan='2'>[[3j9m]] is a 10 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3J9M OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3J9M FirstGlance]. <br> |
| </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=GDP:GUANOSINE-5-DIPHOSPHATE'>GDP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr> | | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.5Å</td></tr> |
| <tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=UNK:UNKNOWN'>UNK</scene></td></tr> | | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=GDP:GUANOSINE-5-DIPHOSPHATE'>GDP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr> |
| <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3j9m FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3j9m OCA], [http://www.rcsb.org/pdb/explore.do?structureId=3j9m RCSB], [http://www.ebi.ac.uk/pdbsum/3j9m PDBsum]</span></td></tr> | | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=3j9m FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3j9m OCA], [https://pdbe.org/3j9m PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3j9m RCSB], [https://www.ebi.ac.uk/pdbsum/3j9m PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3j9m ProSAT]</span></td></tr> |
| </table> | | </table> |
| == Disease ==
| |
| [[http://www.uniprot.org/uniprot/RT22_HUMAN RT22_HUMAN]] Hypotonia with lactic acidemia and hyperammonemia. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/RT16_HUMAN RT16_HUMAN]] Combined oxidative phosphorylation defect type 2. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/RM03_HUMAN RM03_HUMAN]] Combined oxidative phosphorylation defect type 9. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/RM44_HUMAN RM44_HUMAN]] Infantile hypertrophic cardiomyopathy due to MRPL44 deficiency. The disease is caused by mutations affecting the gene represented in this entry.
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| == Function == | | == Function == |
| [[http://www.uniprot.org/uniprot/AKIP_HUMAN AKIP_HUMAN]] May act as a negative regulator of Aurora-A kinase, by down-regulation through proteasome-dependent degradation. [[http://www.uniprot.org/uniprot/RM14_HUMAN RM14_HUMAN]] Forms part of 2 intersubunit bridges in the assembled ribosome. Upon binding to MALSU1 intersubunit bridge formation is blocked, preventing ribosome formation and repressing translation (Probable).<ref>PMID:22829778</ref> [[http://www.uniprot.org/uniprot/RT29_HUMAN RT29_HUMAN]] Involved in mediating interferon-gamma-induced cell death. [[http://www.uniprot.org/uniprot/RM36_HUMAN RM36_HUMAN]] Component of the large subunit of the mitochondrial ribosome. [[http://www.uniprot.org/uniprot/G45IP_HUMAN G45IP_HUMAN]] Acts as a negative regulator of G1 to S cell cycle phase progression by inhibiting cyclin-dependent kinases. Inhibitory effects are additive with GADD45 proteins but occurs also in the absence of GADD45 proteins. Acts as a repressor of the orphan nuclear receptor NR4A1 by inhibiting AB domain-mediated transcriptional activity. May be involved in the hormone-mediated regulation of NR4A1 transcriptional activity. May play a role in mitochondrial protein synthesis. [[http://www.uniprot.org/uniprot/RM16_HUMAN RM16_HUMAN]] Component of the large subunit of mitochondrial ribosome. [[http://www.uniprot.org/uniprot/ICT1_HUMAN ICT1_HUMAN]] Essential peptidyl-tRNA hydrolase component of the mitochondrial large ribosomal subunit. Acts as a codon-independent translation release factor that has lost all stop codon specificity and directs the termination of translation in mitochondrion, possibly in case of abortive elongation. May be involved in the hydrolysis of peptidyl-tRNAs that have been prematurely terminated and thus in the recycling of stalled mitochondrial ribosomes.<ref>PMID:20186120</ref> [[http://www.uniprot.org/uniprot/PTCD3_HUMAN PTCD3_HUMAN]] Mitochondrial RNA-binding protein that has a role in mitochondrial translation.<ref>PMID:19427859</ref> [[http://www.uniprot.org/uniprot/RM41_HUMAN RM41_HUMAN]] Component of the mitochondrial ribosome large subunit. Also involved in apoptosis and cell cycle. Enhances p53/TP53 stability, thereby contributing to p53/TP53-induced apoptosis in response to growth-inhibitory condition. Enhances p53/TP53 translocation to the mitochondria. Has the ability to arrest the cell cycle at the G1 phase, possibly by stabilizing the CDKN1A and CDKN1B (p27Kip1) proteins.<ref>PMID:16024796</ref> <ref>PMID:16256947</ref> [[http://www.uniprot.org/uniprot/RM44_HUMAN RM44_HUMAN]] Component of the 39S subunit of mitochondrial ribosome. May have a function in the assembly/stability of nascent mitochondrial polypeptides exiting the ribosome.<ref>PMID:23315540</ref> | | [https://www.uniprot.org/uniprot/RM42_HUMAN RM42_HUMAN] |
| <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
| == Publication Abstract from PubMed == | | == Publication Abstract from PubMed == |
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| From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> | | From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br> |
| </div> | | </div> |
| | <div class="pdbe-citations 3j9m" style="background-color:#fffaf0;"></div> |
| | |
| | ==See Also== |
| | *[[Ribosome 3D structures|Ribosome 3D structures]] |
| | *[[Transfer RNA (tRNA)|Transfer RNA (tRNA)]] |
| == References == | | == References == |
| <references/> | | <references/> |
| __TOC__ | | __TOC__ |
| </StructureSection> | | </SX> |
| [[Category: Homo sapiens]] | | [[Category: Homo sapiens]] |
| [[Category: Amunts, A]] | | [[Category: Large Structures]] |
| [[Category: Brown, A]] | | [[Category: Amunts A]] |
| [[Category: Ramakrishnan, V]] | | [[Category: Brown A]] |
| [[Category: Scheres, S H]] | | [[Category: Ramakrishnan V]] |
| [[Category: Toots, J]] | | [[Category: Scheres SH]] |
| [[Category: Mitochondria]]
| | [[Category: Toots J]] |
| [[Category: Ribosome]]
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| [[Category: Translation]]
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