8gs8

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'''Unreleased structure'''
 
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The entry 8gs8 is ON HOLD until Paper Publication
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==cryo-EM structure of the human respiratory complex II==
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<StructureSection load='8gs8' size='340' side='right'caption='[[8gs8]], [[Resolution|resolution]] 2.86&Aring;' scene=''>
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== Structural highlights ==
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<table><tr><td colspan='2'>[[8gs8]] is a 4 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=8GS8 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8GS8 FirstGlance]. <br>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=F3S:FE3-S4+CLUSTER'>F3S</scene>, <scene name='pdbligand=FAD:FLAVIN-ADENINE+DINUCLEOTIDE'>FAD</scene>, <scene name='pdbligand=FES:FE2/S2+(INORGANIC)+CLUSTER'>FES</scene>, <scene name='pdbligand=HEM:PROTOPORPHYRIN+IX+CONTAINING+FE'>HEM</scene>, <scene name='pdbligand=PEV:(1S)-2-{[(2-AMINOETHOXY)(HYDROXY)PHOSPHORYL]OXY}-1-[(PALMITOYLOXY)METHYL]ETHYL+STEARATE'>PEV</scene>, <scene name='pdbligand=SF4:IRON/SULFUR+CLUSTER'>SF4</scene>, <scene name='pdbligand=UQ1:UBIQUINONE-1'>UQ1</scene></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=8gs8 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8gs8 OCA], [https://pdbe.org/8gs8 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8gs8 RCSB], [https://www.ebi.ac.uk/pdbsum/8gs8 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8gs8 ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/SDHA_HUMAN SDHA_HUMAN] Isolated succinate-CoQ reductase deficiency;Leigh syndrome with leukodystrophy;Hereditary pheochromocytoma-paraganglioma;Familial isolated dilated cardiomyopathy;Gastrointestinal stromal tumor. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry. The disease is caused by variants affecting the gene represented in this entry.
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== Function ==
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[https://www.uniprot.org/uniprot/SDHA_HUMAN SDHA_HUMAN] Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:24781757). Can act as a tumor suppressor (PubMed:20484225).<ref>PMID:20484225</ref> <ref>PMID:24781757</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Human complex II is a key protein complex that links two essential energy-producing processes: the tricarboxylic acid cycle and oxidative phosphorylation. Deficiencies due to mutagenesis have been shown to cause mitochondrial disease and some types of cancers. However, the structure of this complex is yet to be resolved, hindering a comprehensive understanding of the functional aspects of this molecular machine. Here, we have determined the structure of human complex II in the presence of ubiquinone at 2.86 A resolution by cryoelectron microscopy, showing it comprises two water-soluble subunits, SDHA and SDHB, and two membrane-spanning subunits, SDHC and SDHD. This structure allows us to propose a route for electron transfer. In addition, clinically relevant mutations are mapped onto the structure. This mapping provides a molecular understanding to explain why these variants have the potential to produce disease.
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Authors: Du, Z., Zhou, X., Lai, Y., Xu, J., Zhang, Y., Zhou, S., Liu, F., Gao, Y., Gong, H., Rao, Z.
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Structure of the human respiratory complex II.,Du Z, Zhou X, Lai Y, Xu J, Zhang Y, Zhou S, Feng Z, Yu L, Tang Y, Wang W, Yu L, Tian C, Ran T, Chen H, Guddat LW, Liu F, Gao Y, Rao Z, Gong H Proc Natl Acad Sci U S A. 2023 May 2;120(18):e2216713120. doi: , 10.1073/pnas.2216713120. Epub 2023 Apr 25. PMID:37098072<ref>PMID:37098072</ref>
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Description: cryo-EM structure of the human respiratory complex II
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[Category: Unreleased Structures]]
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</div>
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[[Category: Gong, H]]
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<div class="pdbe-citations 8gs8" style="background-color:#fffaf0;"></div>
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[[Category: Gao, Y]]
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== References ==
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[[Category: Zhou, X]]
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<references/>
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[[Category: Du, Z]]
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__TOC__
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[[Category: Rao, Z]]
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</StructureSection>
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[[Category: Lai, Y]]
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[[Category: Homo sapiens]]
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[[Category: Zhang, Y]]
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[[Category: Large Structures]]
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[[Category: Liu, F]]
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[[Category: Du Z]]
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[[Category: Xu, J]]
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[[Category: Gao Y]]
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[[Category: Zhou, S]]
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[[Category: Gong H]]
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[[Category: Lai Y]]
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[[Category: Liu F]]
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[[Category: Rao Z]]
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[[Category: Xu J]]
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[[Category: Zhang Y]]
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[[Category: Zhou S]]
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[[Category: Zhou X]]

Revision as of 06:57, 10 May 2023

cryo-EM structure of the human respiratory complex II

PDB ID 8gs8

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