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Line 3: |
| | <StructureSection load='6bzr' size='340' side='right'caption='[[6bzr]], [[Resolution|resolution]] 2.80Å' scene=''> | | <StructureSection load='6bzr' size='340' side='right'caption='[[6bzr]], [[Resolution|resolution]] 2.80Å' scene=''> |
| | == Structural highlights == | | == Structural highlights == |
| - | <table><tr><td colspan='2'>[[6bzr]] is a 2 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=6BZR OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6BZR FirstGlance]. <br> | + | <table><tr><td colspan='2'>[[6bzr]] is a 2 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=6BZR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6BZR FirstGlance]. <br> |
| - | </td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=CIT:CITRIC+ACID'>CIT</scene></td></tr> | + | </td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 2.7999945Å</td></tr> |
| - | <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='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ADP:ADENOSINE-5-DIPHOSPHATE'>ADP</scene>, <scene name='pdbligand=CIT:CITRIC+ACID'>CIT</scene></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=6bzr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bzr OCA], [http://pdbe.org/6bzr PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6bzr RCSB], [http://www.ebi.ac.uk/pdbsum/6bzr PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6bzr ProSAT]</span></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=6bzr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6bzr OCA], [https://pdbe.org/6bzr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6bzr RCSB], [https://www.ebi.ac.uk/pdbsum/6bzr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6bzr ProSAT]</span></td></tr> |
| | </table> | | </table> |
| | == Disease == | | == Disease == |
| - | [[http://www.uniprot.org/uniprot/MRP6_HUMAN MRP6_HUMAN]] Generalized arterial calcification of infancy;Pseudoxanthoma elasticum. The disease is caused by mutations affecting the gene represented in this entry. Homozygous or compound heterozygous ABCC6 mutations have been found in the overwhelming majority of cases. Individuals carrying heterozygous mutations express limited manifestations of the pseudoxanthoma elasticum phenotype. The disease is caused by mutations affecting the gene represented in this entry. | + | [https://www.uniprot.org/uniprot/MRP6_HUMAN MRP6_HUMAN] Generalized arterial calcification of infancy;Pseudoxanthoma elasticum. The disease is caused by mutations affecting the gene represented in this entry. Homozygous or compound heterozygous ABCC6 mutations have been found in the overwhelming majority of cases. Individuals carrying heterozygous mutations express limited manifestations of the pseudoxanthoma elasticum phenotype. The disease is caused by mutations affecting the gene represented in this entry. |
| | == 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> | + | [https://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> |
| | == References == | | == References == |
| | <references/> | | <references/> |
| | __TOC__ | | __TOC__ |
| | </StructureSection> | | </StructureSection> |
| - | [[Category: Human]] | + | [[Category: Homo sapiens]] |
| | [[Category: Large Structures]] | | [[Category: Large Structures]] |
| - | [[Category: Thibodeau, P H]] | + | [[Category: Thibodeau PH]] |
| - | [[Category: Zheng, A]] | + | [[Category: Zheng A]] |
| - | [[Category: Abcc6]]
| + | |
| - | [[Category: Adp-bound state]]
| + | |
| - | [[Category: Atp-binding cassette transporter c6]]
| + | |
| - | [[Category: Nbd2]]
| + | |
| - | [[Category: Nucleotide binding domain 2]]
| + | |
| - | [[Category: Transport protein]]
| + | |
| Structural highlights
Disease
MRP6_HUMAN Generalized arterial calcification of infancy;Pseudoxanthoma elasticum. The disease is caused by mutations affecting the gene represented in this entry. Homozygous or compound heterozygous ABCC6 mutations have been found in the overwhelming majority of cases. Individuals carrying heterozygous mutations express limited manifestations of the pseudoxanthoma elasticum phenotype. The disease is caused by mutations affecting the gene represented in this entry.
Function
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).[1] Isoform 2: Inhibits TNF-alpha-mediated apoptosis through blocking one or more caspases.[2]
References
- ↑ Ilias A, Urban Z, Seidl TL, Le Saux O, Sinko E, Boyd CD, Sarkadi B, Varadi A. Loss of ATP-dependent transport activity in pseudoxanthoma elasticum-associated mutants of human ABCC6 (MRP6). J Biol Chem. 2002 May 10;277(19):16860-7. doi: 10.1074/jbc.M110918200. Epub 2002 , Mar 5. PMID:11880368 doi:http://dx.doi.org/10.1074/jbc.M110918200
- ↑ Ostuni A, Lara P, Armentano MF, Miglionico R, Salvia AM, Monnich M, Carmosino M, Lasorsa FM, Monne M, Nilsson I, Bisaccia F. The hepatitis B x antigen anti-apoptotic effector URG7 is localized to the endoplasmic reticulum membrane. FEBS Lett. 2013 Sep 17;587(18):3058-62. doi: 10.1016/j.febslet.2013.07.042. Epub , 2013 Jul 31. PMID:23912081 doi:http://dx.doi.org/10.1016/j.febslet.2013.07.042
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