5va2 - Revision history

OCA at 14:10, 13 March 2024

OCA — Wed, 13 Mar 2024 14:10:07 GMT

←Older revision		Revision as of 14:10, 13 March 2024
Line 3:		Line 3:
	<SX load='5va2' size='340' side='right' viewer='molstar' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>		<SX load='5va2' size='340' side='right' viewer='molstar' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5va2]] 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=5VA2 OCA]. For a <b>guided tour on the structure components</b> use [~~http~~://proteopedia.org/fgij/fg.htm?mol=5VA2 FirstGlance]. <br>	+	<table><tr><td colspan='2'>[[5va2]] is a 1 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=5VA2 OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/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>~~	+	</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models\|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution\|Resolution]] 3.8Å</td></tr>
-	~~<tr~~ id~~='gene'><td class~~="~~sblockLbl~~">~~<b>~~[[~~Gene~~\|~~Gene:~~]]~~</b></td><td class="sblockDat">KCNH2, ERG, ERG1, HERG ([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'>[https://proteopedia.org/fgij/fg.htm?mol=5va2 FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5va2 OCA], [https://pdbe.org/5va2 PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5va2 RCSB], [https://www.ebi.ac.uk/pdbsum/5va2 PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5va2 ProSAT]</span></td></tr>
-	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[~~http~~://proteopedia.org/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>		</table>
	== Disease ==		== 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>	+	[https://www.uniprot.org/uniprot/KCNH2_HUMAN KCNH2_HUMAN] Defects in KCNH2 are the cause of long QT syndrome type 2 (LQT2) [MIM:[https://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:[https://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 ==		== 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.	+	[https://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.	+
-		+
-	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>	+
-		+
-	~~From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine.<br>~~	+
-	~~</div>~~	+
-	~~<div class="pdbe-citations 5va2" style="background-color:#fffaf0;"></div>~~	+

	==See Also==		==See Also==
Line 28:		Line 18:
	__TOC__		__TOC__
	</SX>		</SX>
-	[[Category: ~~Human~~]]	+	[[Category: Homo sapiens]]
	[[Category: Large Structures]]		[[Category: Large Structures]]
-	[[Category: MacKinnon, R]]	+	[[Category: MacKinnon R]]
-	[[Category: Wang~~, W W]]~~	+	[[Category: Wang WW]]
-	~~[[Category: Cnbhd]]~~	+
-	~~[[Category: K+ channel]]~~	+
-	~~[[Category: Pa]]~~	+
-	~~[[Category: Selectivity filter]]~~	+
-	~~[[Category: Transport protein]]~~	+
-	~~[[Category: Voltage sensor~~]]	+

OCA at 22:02, 10 April 2020

OCA — Fri, 10 Apr 2020 22:02:46 GMT

←Older revision		Revision as of 22:02, 10 April 2020
Line 3:		Line 3:
	<SX load='5va2' size='340' side='right' viewer='molstar' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>		<SX load='5va2' size='340' side='right' viewer='molstar' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
-	<table><tr><td colspan='2'>[[5va2]] 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=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>	+	<table><tr><td colspan='2'>[[5va2]] 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=5VA2 OCA]. For a <b>guided tour on the structure components</b> use [http://proteopedia.org/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>		</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='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">KCNH2, ERG, ERG1, HERG ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>		<tr id='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">KCNH2, ERG, ERG1, HERG ([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=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>	+	<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[http://proteopedia.org/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>		</table>
	== Disease ==		== Disease ==

OCA at 19:49, 6 March 2020

OCA — Fri, 06 Mar 2020 19:49:41 GMT

←Older revision		Revision as of 19:49, 6 March 2020
Line 1:		Line 1:

	==Cryo-EM structure of the human ether-a-go-go related K+ channel==		==Cryo-EM structure of the human ether-a-go-go related K+ channel==
-	<~~StructureSection~~ load='5va2' size='340' side='right'caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>	+	<SX load='5va2' size='340' side='right' viewer='molstar' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[5va2]] 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=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>		<table><tr><td colspan='2'>[[5va2]] 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=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>
Line 23:		Line 23:

	==See Also==		==See Also==
-	*[[Potassium ~~Channel~~\|Potassium ~~Channel~~]]	+	*[[Potassium channel 3D structures\|Potassium channel 3D structures]]
	== References ==		== References ==
	<references/>		<references/>
	__TOC__		__TOC__
-	</~~StructureSection~~>	+	</SX>
	[[Category: Human]]		[[Category: Human]]
	[[Category: Large Structures]]		[[Category: Large Structures]]

OCA at 16:54, 20 November 2019

OCA — Wed, 20 Nov 2019 16:54:25 GMT

←Older revision		Revision as of 16:54, 20 November 2019
Line 1:		Line 1:

	==Cryo-EM structure of the human ether-a-go-go related K+ channel==		==Cryo-EM structure of the human ether-a-go-go related K+ channel==
-	<StructureSection load='5va2' size='340' side='right' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>	+	<StructureSection load='5va2' size='340' side='right'caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>
	== Structural highlights ==		== Structural highlights ==
	<table><tr><td colspan='2'>[[5va2]] 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=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>		<table><tr><td colspan='2'>[[5va2]] 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=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>
Line 29:		Line 29:
	</StructureSection>		</StructureSection>
	[[Category: Human]]		[[Category: Human]]
		+	[[Category: Large Structures]]
	[[Category: MacKinnon, R]]		[[Category: MacKinnon, R]]
	[[Category: Wang, W W]]		[[Category: Wang, W W]]

OCA at 09:22, 18 July 2018

OCA — Wed, 18 Jul 2018 09:22:31 GMT

←Older revision		Revision as of 09:22, 18 July 2018
Line 3:		Line 3:
	<StructureSection load='5va2' size='340' side='right' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>		<StructureSection load='5va2' size='340' side='right' caption='[[5va2]], [[Resolution\|resolution]] 3.80Å' scene=''>
	== Structural highlights ==		== 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>	+	<table><tr><td colspan='2'>[[5va2]] 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=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>		</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='gene'><td class="sblockLbl"><b>[[Gene\|Gene:]]</b></td><td class="sblockDat">KCNH2, ERG, ERG1, HERG ([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=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>		<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>		</table>
Line 27:		Line 28:
	__TOC__		__TOC__
	</StructureSection>		</StructureSection>
		+	[[Category: Human]]
	[[Category: MacKinnon, R]]		[[Category: MacKinnon, R]]
	[[Category: Wang, W W]]		[[Category: Wang, W W]]

OCA at 08:31, 6 October 2017

OCA — Fri, 06 Oct 2017 08:31:54 GMT

←Older revision		Revision as of 08:31, 6 October 2017
Line 20:		Line 20:
	</div>		</div>
	<div class="pdbe-citations 5va2" style="background-color:#fffaf0;"></div>		<div class="pdbe-citations 5va2" style="background-color:#fffaf0;"></div>
		+
		+	==See Also==
		+	*[[Potassium Channel\|Potassium Channel]]
	== References ==		== References ==
	<references/>		<references/>

OCA at 13:01, 4 May 2017

OCA — Thu, 04 May 2017 13:01:38 GMT

←Older revision		Revision as of 13:01, 4 May 2017
Line 1:		Line 1:
-	'''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]] 3.80Å' 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®/PubMed®, 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]]

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OCA: New page: '''Unreleased structure''' The entry 5va2 is ON HOLD Authors: Wang, W.W., MacKinnon, R. Description: Cryo-EM structure of the human ether-a-go-go related K+ channel [[Category: Unrelea...

OCA — Wed, 05 Apr 2017 11:50:42 GMT

New page: '''Unreleased structure''' The entry 5va2 is ON HOLD Authors: Wang, W.W., MacKinnon, R. Description: Cryo-EM structure of the human ether-a-go-go related K+ channel [[Category: Unrelea...

New page

'''Unreleased structure'''

The entry 5va2 is ON HOLD

Authors: Wang, W.W., MacKinnon, R.

Description: Cryo-EM structure of the human ether-a-go-go related K+ channel
[[Category: Unreleased Structures]]
[[Category: Wang, W.W]]
[[Category: Mackinnon, R]]