2l4r

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{{STRUCTURE_2l4r| PDB=2l4r | SCENE= }}
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==NMR solution structure of the N-terminal PAS domain of hERG==
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===NMR solution structure of the N-terminal PAS domain of hERG===
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<StructureSection load='2l4r' size='340' side='right' caption='[[2l4r]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
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{{ABSTRACT_PUBMED_21055387}}
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== Structural highlights ==
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<table><tr><td colspan='2'>[[2l4r]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2L4R OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2L4R FirstGlance]. <br>
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</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 Homo sapiens])</td></tr>
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<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=2l4r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2l4r OCA], [http://www.rcsb.org/pdb/explore.do?structureId=2l4r RCSB], [http://www.ebi.ac.uk/pdbsum/2l4r PDBsum]</span></td></tr>
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</table>
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== Disease ==
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[[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>
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== Function ==
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[[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.
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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The human Ether-a-go-go Related Gene (hERG) potassium channel mediates the rapid delayed rectifier current (IKr) in the cardiac action potential. Mutations in the 135 amino acid residue N-terminal domain (NTD) cause channel dysfunction or mis-translocation. To study the structure of NTD, it was overexpressed and purified from Escherichia coli cells using affinity purification and gel filtration chromatography. The purified protein behaved as a monomer under purification conditions. Far- and near-UV, circular dichroism (CD) and solution nuclear magnetic resonance (NMR) studies showed that the purified protein was well-folded. The solution structure of NTD was obtained and the N-terminal residues 13-23 forming an amphipathic helix which may be important for the protein-protein or protein-membrane interactions. NMR titration experiment also demonstrated that residues from 88 to 94 in NTD are important for the molecular interaction with the peptide derived from the S4-S5 linker.
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==Disease==
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NMR solution structure of the N-terminal domain of hERG and its interaction with the S4-S5 linker.,Li Q, Gayen S, Chen AS, Huang Q, Raida M, Kang C Biochem Biophys Res Commun. 2010 Dec 3;403(1):126-32. Epub 2010 Nov 3. PMID:21055387<ref>PMID:21055387</ref>
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[[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>
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==Function==
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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[[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.
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</div>
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==About this Structure==
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[[2l4r]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2L4R OCA].
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==See Also==
==See Also==
*[[Potassium Channel|Potassium Channel]]
*[[Potassium Channel|Potassium Channel]]
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== References ==
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==Reference==
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<references/>
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<ref group="xtra">PMID:021055387</ref><references group="xtra"/><references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Chen, A S.]]
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[[Category: Chen, A S]]
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[[Category: Gayen, N.]]
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[[Category: Gayen, N]]
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[[Category: Huang, Q.]]
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[[Category: Huang, Q]]
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[[Category: Kang, C.]]
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[[Category: Kang, C]]
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[[Category: Li, Q.]]
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[[Category: Li, Q]]
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[[Category: Raida, M.]]
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[[Category: Raida, M]]
[[Category: Eag domain]]
[[Category: Eag domain]]
[[Category: Herg]]
[[Category: Herg]]

Revision as of 12:17, 18 December 2014

NMR solution structure of the N-terminal PAS domain of hERG

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