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==Crystal Structure of AnkB 24 Ankyrin Repeats in Complex with AnkR Autoinhibition Segment==
==Crystal Structure of AnkB 24 Ankyrin Repeats in Complex with AnkR Autoinhibition Segment==
<StructureSection load='4rlv' size='340' side='right' caption='[[4rlv]], [[Resolution|resolution]] 3.49&Aring;' scene=''>
<StructureSection load='4rlv' size='340' side='right'caption='[[4rlv]], [[Resolution|resolution]] 3.49&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[4rlv]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4RLV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=4RLV FirstGlance]. <br>
<table><tr><td colspan='2'>[[4rlv]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens] and [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=4RLV OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4RLV 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='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 3.4945&#8491;</td></tr>
<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene>, <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">[[4rly|4rly]]</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=4rlv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4rlv OCA], [https://pdbe.org/4rlv PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4rlv RCSB], [https://www.ebi.ac.uk/pdbsum/4rlv PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4rlv 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=4rlv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4rlv OCA], [http://www.rcsb.org/pdb/explore.do?structureId=4rlv RCSB], [http://www.ebi.ac.uk/pdbsum/4rlv PDBsum]</span></td></tr>
</table>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/ANK2_HUMAN ANK2_HUMAN] Romano-Ward syndrome. Long QT syndrome 4 (LQT4) [MIM:[https://omim.org/entry/600919 600919]: A heart disorder characterized by a prolonged QT interval on the ECG and polymorphic ventricular arrhythmias. They cause syncope and sudden death in response to exercise or emotional stress, and can present with a sentinel event of sudden cardiac death in infancy. Long QT syndrome type 4 shows many atypical features compared to classical long QT syndromes, including pronounced sinus bradycardia, polyphasic T waves and atrial fibrillation. Cardiac repolarization defects may be not as severe as in classical LQT syndromes and prolonged QT interval on EKG is not a consistent feature. Note=The disease is caused by mutations affecting the gene represented in this entry.<ref>PMID:12571597</ref> <ref>PMID:15178757</ref>
== Function ==
[https://www.uniprot.org/uniprot/ANK2_HUMAN ANK2_HUMAN] In skeletal muscle, required for proper localization of DMD and DCTN4 and for the formation and/or stability of a special subset of microtubules associated with costameres and neuromuscular junctions (By similarity). Attaches integral membrane proteins to cytoskeletal elements. Also binds to cytoskeletal proteins. Required for coordinate assembly of Na/Ca exchanger, Na/K ATPase and InsP3 receptor at sarcoplasmic reticulum sites in cardiomyocytes. Required for the coordinated expression of the Na/K ATPase, Na/Ca exchanger and beta-2-spectrin (SPTBN1) in the inner segment of rod photoreceptors. Required for expression and targeting of SPTBN1 in neonatal cardiomyocytes and for the regulation of neonatal cardiomyocyte contraction rate.<ref>PMID:12571597</ref>
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
</div>
</div>
<div class="pdbe-citations 4rlv" style="background-color:#fffaf0;"></div>
==See Also==
*[[Ankyrin 3D structures|Ankyrin 3D structures]]
== References ==
== References ==
<references/>
<references/>
__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Wang, C]]
[[Category: Homo sapiens]]
[[Category: Wei, Z]]
[[Category: Large Structures]]
[[Category: Zhang, M]]
[[Category: Mus musculus]]
[[Category: Ank repeat]]
[[Category: Wang C]]
[[Category: Protein-protein interaction]]
[[Category: Wei Z]]
[[Category: Structural protein]]
[[Category: Zhang M]]