2rli

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[[Image:2rli.jpg|left|200px]]
 
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{{Structure
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==Solution structure of Cu(I) human Sco2==
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|PDB= 2rli |SIZE=350|CAPTION= <scene name='initialview01'>2rli</scene>
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<StructureSection load='2rli' size='340' side='right'caption='[[2rli]]' scene=''>
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|SITE=
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== Structural highlights ==
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|LIGAND= <scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene>
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<table><tr><td colspan='2'>[[2rli]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2RLI OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2RLI FirstGlance]. <br>
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|ACTIVITY=
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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|GENE= SCO2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=CU1:COPPER+(I)+ION'>CU1</scene></td></tr>
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|DOMAIN=
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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=2rli FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2rli OCA], [https://pdbe.org/2rli PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2rli RCSB], [https://www.ebi.ac.uk/pdbsum/2rli PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2rli ProSAT]</span></td></tr>
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|RELATEDENTRY=
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</table>
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|RESOURCES=<span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2rli FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2rli OCA], [http://www.ebi.ac.uk/pdbsum/2rli PDBsum], [http://www.rcsb.org/pdb/explore.do?structureId=2rli RCSB]</span>
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== Disease ==
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}}
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[https://www.uniprot.org/uniprot/SCO2_HUMAN SCO2_HUMAN] Defects in SCO2 are the cause of fatal infantile cardioencephalomyopathy with cytochrome c oxidase deficiency (FIC) [MIM:[https://omim.org/entry/604377 604377]. This disease is characterized by hypertrophic cardiomyopathy, lactic acidosis, and gliosis. Heart and skeletal muscle show reductions in cytochrome c oxidase (COX) activity, whereas liver and fibroblasts show mild COX deficiencies.<ref>PMID:10545952</ref> <ref>PMID:10749987</ref> <ref>PMID:11673586</ref>
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== Function ==
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'''Solution structure of Cu(I) human Sco2'''
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[https://www.uniprot.org/uniprot/SCO2_HUMAN SCO2_HUMAN] Acts as a copper chaperone, transporting copper to the Cu(A) site on the cytochrome c oxidase subunit II (COX2).
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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==Overview==
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/rl/2rli_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=2rli ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
Human Sco2 is a mitochondrial membrane-bound protein involved in copper supply for the assembly of cytochrome c oxidase in eukaryotes. Its precise action is not yet understood. We report here a structural and dynamic characterization by NMR of the apo and copper(I) forms of the soluble fragment. The structural and metal binding features of human Cu(I)Sco2 are similar to the more often studied Sco1 homolog, although the dynamic properties and the conformational disorder are quite different when the apo forms and the copper(I)-loaded forms of the two proteins are compared separately. Such differences are accounted for in terms of the different physicochemical properties in strategic protein locations. The misfunction of the known pathogenic mutations is discussed on the basis of the obtained structure.
Human Sco2 is a mitochondrial membrane-bound protein involved in copper supply for the assembly of cytochrome c oxidase in eukaryotes. Its precise action is not yet understood. We report here a structural and dynamic characterization by NMR of the apo and copper(I) forms of the soluble fragment. The structural and metal binding features of human Cu(I)Sco2 are similar to the more often studied Sco1 homolog, although the dynamic properties and the conformational disorder are quite different when the apo forms and the copper(I)-loaded forms of the two proteins are compared separately. Such differences are accounted for in terms of the different physicochemical properties in strategic protein locations. The misfunction of the known pathogenic mutations is discussed on the basis of the obtained structure.
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==Disease==
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A structural characterization of human SCO2.,Banci L, Bertini I, Ciofi-Baffoni S, Gerothanassis IP, Leontari I, Martinelli M, Wang S Structure. 2007 Sep;15(9):1132-40. PMID:17850752<ref>PMID:17850752</ref>
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Known disease associated with this structure: Cardioencephalomyopathy, fatal infantile, due to cytochrome c oxidase deficiency OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=604272 604272]]
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==About this Structure==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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2RLI is a [[Single protein]] structure of 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=2RLI OCA].
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</div>
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<div class="pdbe-citations 2rli" style="background-color:#fffaf0;"></div>
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==Reference==
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== References ==
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A structural characterization of human SCO2., Banci L, Bertini I, Ciofi-Baffoni S, Gerothanassis IP, Leontari I, Martinelli M, Wang S, Structure. 2007 Sep;15(9):1132-40. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/17850752 17850752]
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Banci, L.]]
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[[Category: Banci L]]
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[[Category: Bertini, I.]]
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[[Category: Bertini I]]
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[[Category: Ciofi-baffoni, S.]]
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[[Category: Ciofi-baffoni S]]
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[[Category: Gerothanassis, I P.]]
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[[Category: Gerothanassis IP]]
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[[Category: Leontari, I.]]
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[[Category: Leontari I]]
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[[Category: Martinelli, M.]]
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[[Category: Martinelli M]]
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[[Category: SPINE, Structural Proteomics in Europe.]]
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[[Category: Wang S]]
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[[Category: Wang, S.]]
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[[Category: copper protein]]
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[[Category: metal transport]]
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[[Category: spine]]
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[[Category: spine2-complex]]
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[[Category: structural genomic]]
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[[Category: structural proteomics in europe]]
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[[Category: thioredoxin fold]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Mar 31 05:01:44 2008''
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Current revision

Solution structure of Cu(I) human Sco2

PDB ID 2rli

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OCA

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