6oww

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Current revision (07:18, 11 October 2023) (edit) (undo)
 
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<StructureSection load='6oww' size='340' side='right'caption='[[6oww]], [[Resolution|resolution]] 3.84&Aring;' scene=''>
<StructureSection load='6oww' size='340' side='right'caption='[[6oww]], [[Resolution|resolution]] 3.84&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[6oww]] is a 8 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=6OWW OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/fgij/fg.htm?mol=6OWW FirstGlance]. <br>
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<table><tr><td colspan='2'>[[6oww]] is a 8 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=6OWW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6OWW 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=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=YB:YTTERBIUM+(III)+ION'>YB</scene></td></tr>
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.84&#8491;</td></tr>
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<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[6owv|6owv]]</div></td></tr>
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<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene>, <scene name='pdbligand=YB:YTTERBIUM+(III)+ION'>YB</scene></td></tr>
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">CASQ2 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</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=6oww FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6oww OCA], [https://pdbe.org/6oww PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6oww RCSB], [https://www.ebi.ac.uk/pdbsum/6oww PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6oww ProSAT]</span></td></tr>
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/fgij/fg.htm?mol=6oww FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6oww OCA], [http://pdbe.org/6oww PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6oww RCSB], [http://www.ebi.ac.uk/pdbsum/6oww PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6oww ProSAT]</span></td></tr>
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</table>
</table>
== Disease ==
== Disease ==
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[[http://www.uniprot.org/uniprot/CASQ2_HUMAN CASQ2_HUMAN]] Defects in CASQ2 are the cause of catecholaminergic polymorphic ventricular tachycardia type 2 (CPVT2) [MIM:[http://omim.org/entry/611938 611938]]; also known as stress-induced polymorphic ventricular tachycardia (VTSIP). CPVT2 is an autosomal recessive form of arrhythmogenic disorder characterized by stress-induced, bidirectional ventricular tachycardia that may degenerate into cardiac arrest and cause sudden death.<ref>PMID:17881003</ref> <ref>PMID:11704930</ref> <ref>PMID:15485681</ref> <ref>PMID:16908766</ref> <ref>PMID:18399795</ref>
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[https://www.uniprot.org/uniprot/CASQ2_HUMAN CASQ2_HUMAN] Defects in CASQ2 are the cause of catecholaminergic polymorphic ventricular tachycardia type 2 (CPVT2) [MIM:[https://omim.org/entry/611938 611938]; also known as stress-induced polymorphic ventricular tachycardia (VTSIP). CPVT2 is an autosomal recessive form of arrhythmogenic disorder characterized by stress-induced, bidirectional ventricular tachycardia that may degenerate into cardiac arrest and cause sudden death.<ref>PMID:17881003</ref> <ref>PMID:11704930</ref> <ref>PMID:15485681</ref> <ref>PMID:16908766</ref> <ref>PMID:18399795</ref>
== Function ==
== Function ==
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[[http://www.uniprot.org/uniprot/CASQ2_HUMAN CASQ2_HUMAN]] Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. The release of calcium bound to calsequestrin through a calcium release channel triggers muscle contraction. The skeletal muscle isoform (CASQ1) binds around 80 Ca(2+) ions, while the cardiac isoform (CASQ2) binds approximately 60 Ca(2+) ions.<ref>PMID:17881003</ref>
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[https://www.uniprot.org/uniprot/CASQ2_HUMAN CASQ2_HUMAN] Calsequestrin is a high-capacity, moderate affinity, calcium-binding protein and thus acts as an internal calcium store in muscle. The release of calcium bound to calsequestrin through a calcium release channel triggers muscle contraction. The skeletal muscle isoform (CASQ1) binds around 80 Ca(2+) ions, while the cardiac isoform (CASQ2) binds approximately 60 Ca(2+) ions.<ref>PMID:17881003</ref>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
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[[Category: Human]]
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[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
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[[Category: Deiter, F H]]
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[[Category: Deiter FH]]
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[[Category: Deo, R C]]
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[[Category: Deo RC]]
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[[Category: Jura, N]]
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[[Category: Jura N]]
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[[Category: Shi, C]]
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[[Category: Shi C]]
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[[Category: Titus, E W]]
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[[Category: Titus EW]]
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[[Category: Calcium-binding protein]]
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[[Category: Calsequestrin]]
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[[Category: Metal binding protein]]
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[[Category: Sarcoplasmic reticulum protein]]
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Current revision

Crystal structure of a Human Cardiac Calsequestrin Filament Complexed with Ytterbium

PDB ID 6oww

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