2ew9

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{{STRUCTURE_2ew9| PDB=2ew9 | SCENE= }}
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==Solution structure of apoWLN5-6==
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===Solution structure of apoWLN5-6===
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<StructureSection load='2ew9' size='340' side='right' caption='[[2ew9]], [[NMR_Ensembles_of_Models | 31 NMR models]]' scene=''>
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{{ABSTRACT_PUBMED_16571664}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2ew9]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2EW9 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2EW9 FirstGlance]. <br>
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==Disease==
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</td></tr><tr><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ATP7B, PWD, WC1, WND ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 Homo sapiens])</td></tr>
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[[http://www.uniprot.org/uniprot/ATP7B_HUMAN ATP7B_HUMAN]] Defects in ATP7B are the cause of Wilson disease (WD) [MIM:[http://omim.org/entry/277900 277900]]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.<ref>PMID:8298641</ref><ref>PMID:7626145</ref><ref>PMID:8533760</ref><ref>PMID:8938442</ref><ref>PMID:8931691</ref><ref>PMID:8782057</ref><ref>PMID:9311736</ref><ref>PMID:9772425</ref><ref>PMID:9222767</ref><ref>PMID:8980283</ref><ref>PMID:9887381</ref><ref>PMID:9482578</ref><ref>PMID:9554743</ref><ref>PMID:9452121</ref><ref>PMID:9671269</ref><ref>PMID:9829905</ref><ref>PMID:10194254</ref><ref>PMID:10447265</ref><ref>PMID:10502776</ref><ref>PMID:10502777</ref><ref>PMID:10051024</ref><ref>PMID:10544227</ref><ref>PMID:10453196</ref><ref>PMID:11216666</ref><ref>PMID:11093740</ref><ref>PMID:10790207</ref><ref>PMID:10721669</ref><ref>PMID:11043508</ref><ref>PMID:11180609</ref><ref>PMID:11690702</ref><ref>PMID:11243728</ref><ref>PMID:11954751</ref><ref>PMID:12544487</ref><ref>PMID:12325021</ref><ref>PMID:12376745</ref><ref>PMID:14986826</ref><ref>PMID:14639035</ref><ref>PMID:15024742</ref><ref>PMID:15557537</ref><ref>PMID:14966923</ref><ref>PMID:15845031</ref><ref>PMID:15811015</ref><ref>PMID:15952988</ref><ref>PMID:16207219</ref><ref>PMID:16283883</ref><ref>PMID:16088907</ref><ref>PMID:15967699</ref><ref>PMID:17718866</ref><ref>PMID:18373411</ref><ref>PMID:18203200</ref><ref>PMID:21682854</ref>
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<tr><td class="sblockLbl"><b>Activity:</b></td><td class="sblockDat"><span class='plainlinks'>[http://en.wikipedia.org/wiki/Copper-exporting_ATPase Copper-exporting ATPase], with EC number [http://www.brenda-enzymes.info/php/result_flat.php4?ecno=3.6.3.4 3.6.3.4] </span></td></tr>
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<tr><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2ew9 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2ew9 OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2ew9 RCSB], [http://www.ebi.ac.uk/pdbsum/2ew9 PDBsum]</span></td></tr>
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==Function==
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<table>
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== Disease ==
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[[http://www.uniprot.org/uniprot/ATP7B_HUMAN ATP7B_HUMAN]] Defects in ATP7B are the cause of Wilson disease (WD) [MIM:[http://omim.org/entry/277900 277900]]. WD is an autosomal recessive disorder of copper metabolism in which copper cannot be incorporated into ceruloplasmin in liver, and cannot be excreted from the liver into the bile. Copper accumulates in the liver and subsequently in the brain and kidney. The disease is characterized by neurologic manifestations and signs of cirrhosis.<ref>PMID:8298641</ref> <ref>PMID:7626145</ref> <ref>PMID:8533760</ref> <ref>PMID:8938442</ref> <ref>PMID:8931691</ref> <ref>PMID:8782057</ref> <ref>PMID:9311736</ref> <ref>PMID:9772425</ref> <ref>PMID:9222767</ref> <ref>PMID:8980283</ref> <ref>PMID:9887381</ref> <ref>PMID:9482578</ref> <ref>PMID:9554743</ref> <ref>PMID:9452121</ref> <ref>PMID:9671269</ref> <ref>PMID:9829905</ref> <ref>PMID:10194254</ref> <ref>PMID:10447265</ref> <ref>PMID:10502776</ref> <ref>PMID:10502777</ref> <ref>PMID:10051024</ref> <ref>PMID:10544227</ref> <ref>PMID:10453196</ref> <ref>PMID:11216666</ref> <ref>PMID:11093740</ref> <ref>PMID:10790207</ref> <ref>PMID:10721669</ref> <ref>PMID:11043508</ref> <ref>PMID:11180609</ref> <ref>PMID:11690702</ref> <ref>PMID:11243728</ref> <ref>PMID:11954751</ref> <ref>PMID:12544487</ref> <ref>PMID:12325021</ref> <ref>PMID:12376745</ref> <ref>PMID:14986826</ref> <ref>PMID:14639035</ref> <ref>PMID:15024742</ref> <ref>PMID:15557537</ref> <ref>PMID:14966923</ref> <ref>PMID:15845031</ref> <ref>PMID:15811015</ref> <ref>PMID:15952988</ref> <ref>PMID:16207219</ref> <ref>PMID:16283883</ref> <ref>PMID:16088907</ref> <ref>PMID:15967699</ref> <ref>PMID:17718866</ref> <ref>PMID:18373411</ref> <ref>PMID:18203200</ref> <ref>PMID:21682854</ref>
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== Function ==
[[http://www.uniprot.org/uniprot/ATP7B_HUMAN ATP7B_HUMAN]] Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
[[http://www.uniprot.org/uniprot/ATP7B_HUMAN ATP7B_HUMAN]] Involved in the export of copper out of the cells, such as the efflux of hepatic copper into the bile.
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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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/ew/2ew9_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/chain_selection.php?pdb_ID=2ata 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 ==
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Human Wilson protein is a copper-transporting ATPase located in the secretory pathway possessing six N-terminal metal-binding domains. Here we focus on the function of the metal-binding domains closest to the vesicular portion of the copper pump, i.e., domain 4 (WLN4), and a construct of domains 5 and 6 (WLN5-6). For comparison purposes, some experiments were also performed with domain 2 (WLN2). The solution structure of apoWLN5-6 consists of two ferredoxin folds connected by a short linker, and (15)N relaxation rate measurements show that it behaves as a unit in solution. An NMR titration of apoWLN5-6 with the metallochaperone Cu(I)HAH1 reveals no complex formation and no copper exchange between the two proteins, whereas titration of Cu(I)HAH1 with WLN4 shows the formation of an adduct that is in fast exchange on the NMR time scale with the isolated protein species as confirmed by (15)N relaxation data. A similar interaction is also observed between Cu(I)HAH1 and WLN2; however, the relative amount of the adduct in the protein mixture is lower. An NMR titration of apoWLN5-6 with Cu(I)WLN4 shows copper transfer, first to WLN6 then to WLN5, without the formation of an adduct. Therefore, we suggest that WLN4 and WLN2 are two acceptors of Cu(I) from HAH1, which then somehow route copper to WLN5-6, before the ATP-driven transport of copper across the vesicular membrane.
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==About this Structure==
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Structure of human Wilson protein domains 5 and 6 and their interplay with domain 4 and the copper chaperone HAH1 in copper uptake.,Achila D, Banci L, Bertini I, Bunce J, Ciofi-Baffoni S, Huffman DL Proc Natl Acad Sci U S A. 2006 Apr 11;103(15):5729-34. Epub 2006 Mar 29. PMID:16571664<ref>PMID:16571664</ref>
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[[2ew9]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2EW9 OCA].
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==Reference==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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<ref group="xtra">PMID:016571664</ref><references group="xtra"/><references/>
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</div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Copper-exporting ATPase]]
[[Category: Copper-exporting ATPase]]
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]

Revision as of 02:27, 30 September 2014

Solution structure of apoWLN5-6

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