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| | <SX load='5xtb' size='340' side='right' viewer='molstar' caption='[[5xtb]], [[Resolution|resolution]] 3.40Å' scene=''> | | <SX load='5xtb' size='340' side='right' viewer='molstar' caption='[[5xtb]], [[Resolution|resolution]] 3.40Å' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[5xtb]] is a 18 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=5XTB OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=5XTB FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[5xtb]] 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=5XTB OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5XTB FirstGlance]. <br> |
| - | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=8Q1:S-[2-({N-[(2R)-2-hydroxy-3,3-dimethyl-4-(phosphonooxy)butanoyl]-beta-alanyl}amino)ethyl]+dodecanethioate'>8Q1</scene>, <scene name='pdbligand=FES:FE2/S2+(INORGANIC)+CLUSTER'>FES</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NDP:NADPH+DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NDP</scene>, <scene name='pdbligand=SF4:IRON/SULFUR+CLUSTER'>SF4</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]] 3.4Å</td></tr> |
| - | <tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=5xtb FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5xtb OCA], [http://pdbe.org/5xtb PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5xtb RCSB], [http://www.ebi.ac.uk/pdbsum/5xtb PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5xtb ProSAT]</span></td></tr> | + | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=8Q1:~{S}-[2-[3-[[(2~{R})-3,3-dimethyl-2-oxidanyl-4-phosphonooxy-butanoyl]amino]propanoylamino]ethyl]+dodecanethioate'>8Q1</scene>, <scene name='pdbligand=FES:FE2/S2+(INORGANIC)+CLUSTER'>FES</scene>, <scene name='pdbligand=FMN:FLAVIN+MONONUCLEOTIDE'>FMN</scene>, <scene name='pdbligand=NDP:NADPH+DIHYDRO-NICOTINAMIDE-ADENINE-DINUCLEOTIDE+PHOSPHATE'>NDP</scene></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=5xtb FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5xtb OCA], [https://pdbe.org/5xtb PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5xtb RCSB], [https://www.ebi.ac.uk/pdbsum/5xtb PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5xtb ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | == Disease == | | == Disease == |
| - | [[http://www.uniprot.org/uniprot/NDUS4_HUMAN NDUS4_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUS7_HUMAN NDUS7_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUA9_HUMAN NDUA9_HUMAN]] Isolated NADH-CoQ reductase deficiency. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUS8_HUMAN NDUS8_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUS6_HUMAN NDUS6_HUMAN]] Isolated NADH-CoQ reductase deficiency. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUS1_HUMAN NDUS1_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUS3_HUMAN NDUS3_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. [[http://www.uniprot.org/uniprot/NDUV2_HUMAN NDUV2_HUMAN]] Isolated NADH-CoQ reductase deficiency. [[http://www.uniprot.org/uniprot/NDUV1_HUMAN NDUV1_HUMAN]] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. [[http://www.uniprot.org/uniprot/NDUAD_HUMAN NDUAD_HUMAN]] Papillary or follicular thyroid carcinoma. Disease susceptibility is associated with variations affecting the gene represented in this entry. Defects in NDUFA13 are a cause of a mitochondrial complex I deficiency characterized by early onset hypotonia, dyskinesia and sensorial deficiencies, as well as a severe optic neuropathy.<ref>PMID:25901006</ref> [[http://www.uniprot.org/uniprot/NDUAC_HUMAN NDUAC_HUMAN]] The disease is caused by mutations affecting the gene represented in this entry. | + | [https://www.uniprot.org/uniprot/NDUV1_HUMAN NDUV1_HUMAN] Isolated NADH-CoQ reductase deficiency;Leigh syndrome with leukodystrophy. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. |
| | == Function == | | == Function == |
| - | [[http://www.uniprot.org/uniprot/ACPM_HUMAN ACPM_HUMAN]] Carrier of the growing fatty acid chain in fatty acid biosynthesis in mitochondria. Accessory and non-catalytic subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), which functions in the transfer of electrons from NADH to the respiratory chain (By similarity). [[http://www.uniprot.org/uniprot/NDUS4_HUMAN NDUS4_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:12611891</ref> <ref>PMID:9463323</ref> [[http://www.uniprot.org/uniprot/NDUS7_HUMAN NDUS7_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:12611891</ref> [[http://www.uniprot.org/uniprot/NDUA5_HUMAN NDUA5_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUA9_HUMAN NDUA9_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:22114105</ref> <ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUS8_HUMAN NDUS8_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). May donate electrons to ubiquinone. [[http://www.uniprot.org/uniprot/NDUS6_HUMAN NDUS6_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUS1_HUMAN NDUS1_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. [[http://www.uniprot.org/uniprot/NDUS3_HUMAN NDUS3_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). [[http://www.uniprot.org/uniprot/NDUV2_HUMAN NDUV2_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). [[http://www.uniprot.org/uniprot/NDUA7_HUMAN NDUA7_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUA6_HUMAN NDUA6_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed to be not involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUV1_HUMAN NDUV1_HUMAN]] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). [[http://www.uniprot.org/uniprot/NDUAD_HUMAN NDUAD_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis (PubMed:27626371). Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (PubMed:27626371). Involved in the interferon/all-trans-retinoic acid (IFN/RA) induced cell death. This apoptotic activity is inhibited by interaction with viral IRF1. Prevents the transactivation of STAT3 target genes. May play a role in CARD15-mediated innate mucosal responses and serve to regulate intestinal epithelial cell responses to microbes (PubMed:15753091).<ref>PMID:12628925</ref> <ref>PMID:12867595</ref> <ref>PMID:15753091</ref> <ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUV3_HUMAN NDUV3_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. May be the terminally assembled subunit of Complex I.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUAC_HUMAN NDUAC_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone.<ref>PMID:27626371</ref> [[http://www.uniprot.org/uniprot/NDUA2_HUMAN NDUA2_HUMAN]] Accessory subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I), that is believed not to be involved in catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. | + | [https://www.uniprot.org/uniprot/NDUV1_HUMAN NDUV1_HUMAN] Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). |
| | <div style="background-color:#fffaf0;"> | | <div style="background-color:#fffaf0;"> |
| | == Publication Abstract from PubMed == | | == Publication Abstract from PubMed == |
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| | [[Category: Homo sapiens]] | | [[Category: Homo sapiens]] |
| | [[Category: Large Structures]] | | [[Category: Large Structures]] |
| - | [[Category: Gu, J]] | + | [[Category: Gu J]] |
| - | [[Category: Wu, M]] | + | [[Category: Wu M]] |
| - | [[Category: Yang, M]] | + | [[Category: Yang M]] |
| - | [[Category: Oxidoreductase-electron transport complex]]
| + | |
| - | [[Category: Respiratory]]
| + | |
