5va2: Difference between revisions

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-'''Unreleased structure'''
-The entry 5va2 is ON HOLD
+==Cryo-EM structure of the human ether-a-go-go related K+ channel==
+<StructureSection load='5va2' size='340' side='right' caption='[[5va2]], [[Resolution|resolution]] 3.80&Aring;' scene=''>
+== Structural highlights ==
+<table><tr><td colspan='2'>[[5va2]] is a 1 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5VA2 OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5VA2 FirstGlance]. <br>
+</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[5va1|5va1]], [[5va3|5va3]]</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=5va2 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5va2 OCA], [http://pdbe.org/5va2 PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5va2 RCSB], [http://www.ebi.ac.uk/pdbsum/5va2 PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5va2 ProSAT]</span></td></tr>
+</table>
+== Disease ==
+[[http://www.uniprot.org/uniprot/KCNH2_HUMAN KCNH2_HUMAN]] Defects in KCNH2 are the cause of long QT syndrome type 2 (LQT2) [MIM:[http://omim.org/entry/613688 613688]]. Long QT syndromes are heart disorders 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. Deafness is often associated with LQT2.<ref>PMID:16361248</ref> <ref>PMID:9600240</ref> <ref>PMID:7889573</ref> <ref>PMID:8914737</ref> <ref>PMID:8635257</ref> <ref>PMID:8877771</ref> <ref>PMID:9024139</ref> <ref>PMID:9693036</ref> <ref>PMID:9544837</ref> <ref>PMID:9452080</ref> <ref>PMID:10086971</ref> <ref>PMID:10220144</ref> <ref>PMID:10187793</ref> <ref>PMID:10517660</ref> <ref>PMID:10735633</ref> <ref>PMID:10973849</ref> <ref>PMID:10862094</ref> <ref>PMID:10753933</ref> <ref>PMID:12062363</ref> <ref>PMID:12354768</ref> <ref>PMID:12621127</ref> <ref>PMID:15051636</ref> <ref>PMID:15840476</ref> <ref>PMID:22314138</ref>   Defects in KCNH2 are the cause of short QT syndrome type 1 (SQT1) [MIM:[http://omim.org/entry/609620 609620]]. Short QT syndromes are heart disorders characterized by idiopathic persistently and uniformly short QT interval on ECG in the absence of structural heart disease in affected individuals. They cause syncope and sudden death.<ref>PMID:14676148</ref> <ref>PMID:15828882</ref>
+== Function ==
+[[http://www.uniprot.org/uniprot/KCNH2_HUMAN KCNH2_HUMAN]] Pore-forming (alpha) subunit of voltage-gated inwardly rectifying potassium channel. Channel properties are modulated by cAMP and subunit assembly. Mediates the rapidly activating component of the delayed rectifying potassium current in heart (IKr). Isoform 3 has no channel activity by itself, but modulates channel characteristics when associated with isoform 1.
+<div style="background-color:#fffaf0;">
+== Publication Abstract from PubMed ==
+The human ether-a-go-go-related potassium channel (hERG, Kv11.1) is a voltage-dependent channel known for its role in repolarizing the cardiac action potential. hERG alteration by mutation or pharmacological inhibition produces Long QT syndrome and the lethal cardiac arrhythmia torsade de pointes. We have determined the molecular structure of hERG to 3.8 A using cryo-electron microscopy. In this structure, the voltage sensors adopt a depolarized conformation, and the pore is open. The central cavity has an atypically small central volume surrounded by four deep hydrophobic pockets, which may explain hERG's unusual sensitivity to many drugs. A subtle structural feature of the hERG selectivity filter might correlate with its fast inactivation rate, which is key to hERG's role in cardiac action potential repolarization.
-Authors: Wang, W.W., MacKinnon, R.
+Cryo-EM Structure of the Open Human Ether-a-go-go-Related K+ Channel hERG.,Wang W, MacKinnon R Cell. 2017 Apr 20;169(3):422-430.e10. doi: 10.1016/j.cell.2017.03.048. PMID:28431243<ref>PMID:28431243</ref>
-Description: Cryo-EM structure of the human ether-a-go-go related K+ channel
+From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-[[Category: Unreleased Structures]]
+</div>
-[[Category: Wang, W.W]]
+<div class="pdbe-citations 5va2" style="background-color:#fffaf0;"></div>
-[[Category: Mackinnon, R]]
+== References ==
+<references/>
+__TOC__
+</StructureSection>
+[[Category: MacKinnon, R]]
+[[Category: Wang, W W]]
+[[Category: Cnbhd]]
+[[Category: K+ channel]]
+[[Category: Pa]]
+[[Category: Selectivity filter]]
+[[Category: Transport protein]]
+[[Category: Voltage sensor]]