6bzs

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<StructureSection load='6bzs' size='340' side='right' caption='[[6bzs]], [[Resolution|resolution]] 2.30&Aring;' scene=''>
<StructureSection load='6bzs' size='340' side='right' caption='[[6bzs]], [[Resolution|resolution]] 2.30&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6bzs]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6BZS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6BZS FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6bzs]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6BZS OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6BZS FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6bzr|6bzr]]</td></tr>
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6bzr|6bzr]]</td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">ABCC6, ARA, MRP6 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=6bzs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bzs OCA], [http://pdbe.org/6bzs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6bzs RCSB], [http://www.ebi.ac.uk/pdbsum/6bzs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6bzs ProSAT]</span></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=6bzs FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bzs OCA], [http://pdbe.org/6bzs PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6bzs RCSB], [http://www.ebi.ac.uk/pdbsum/6bzs PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6bzs ProSAT]</span></td></tr>
</table>
</table>
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== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/MRP6_HUMAN MRP6_HUMAN]] Isoform 1: May participate directly in the active transport of drugs into subcellular organelles or influence drug distribution indirectly. Transports glutathione conjugates as leukotriene-c4 (LTC4) and N-ethylmaleimide S-glutathione (NEM-GS).<ref>PMID:11880368</ref> Isoform 2: Inhibits TNF-alpha-mediated apoptosis through blocking one or more caspases.<ref>PMID:23912081</ref>
[[http://www.uniprot.org/uniprot/MRP6_HUMAN MRP6_HUMAN]] Isoform 1: May participate directly in the active transport of drugs into subcellular organelles or influence drug distribution indirectly. Transports glutathione conjugates as leukotriene-c4 (LTC4) and N-ethylmaleimide S-glutathione (NEM-GS).<ref>PMID:11880368</ref> Isoform 2: Inhibits TNF-alpha-mediated apoptosis through blocking one or more caspases.<ref>PMID:23912081</ref>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Mutations in ATP-binding cassette subfamily C member 6 (ABCC6) transporter are associated with pseudoxanthoma elasticum (PXE), a disease resulting in ectopic mineralization and affecting multiple tissues. A growing number of mutations have been identified in individuals with PXE. For most of these variants, no mechanistic information is available regarding their role in normal and pathophysiologies. To assess how PXE-associated mutations alter ABCC6 biosynthesis and structure, we biophysically and biochemically evaluated the N-terminal nucleotide-binding domain of ABCC6. A high-resolution X-ray structure of nucleotide-binding domain 1 (NBD1) of human ABCC6 was obtained at 2.3 A that provided a template on which to evaluate PXE-causing mutations. Biochemical analysis of mutations in this domain indicated that multiple PXE-causing mutations altered its structural properties. Analyses of the full-length protein revealed a strong correlation between the alterations in NBD properties and the processing and expression of ABCC6. These results suggest that a significant fraction of PXE-associated mutations located in NBD1 causes changes in its structural properties and that these mutation-induced alterations directly affect the maturation of the full-length ABCC6 protein.
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Structural analysis reveals pathomechanisms associated with pseudoxanthoma elasticum-causing mutations in the ABCC6 transporter.,Ran Y, Zheng A, Thibodeau PH J Biol Chem. 2018 Aug 28. pii: RA118.004806. doi: 10.1074/jbc.RA118.004806. PMID:30154241<ref>PMID:30154241</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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<div class="pdbe-citations 6bzs" style="background-color:#fffaf0;"></div>
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
[[Category: Thibodeau, P H]]
[[Category: Thibodeau, P H]]
[[Category: Zheng, A]]
[[Category: Zheng, A]]

Revision as of 20:16, 19 September 2018

Human ABCC6 NBD1 in Apo state

6bzs, resolution 2.30Å

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