1ujl: Difference between revisions

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-[[Image:1ujl.gif|left|200px]]<br /><applet load="1ujl" size="350" color="white" frame="true" align="right" spinBox="true"
-caption="1ujl" />
-'''Solution Structure of the HERG K+ channel S5-P extracellular linker'''<br />
-==Overview==
+==Solution Structure of the HERG K+ channel S5-P extracellular linker==
+<StructureSection load='1ujl' size='340' side='right'caption='[[1ujl]]' scene=''>
+== Structural highlights ==
+<table><tr><td colspan='2'>[[1ujl]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1UJL OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1UJL FirstGlance]. <br>
+</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</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=1ujl FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1ujl OCA], [https://pdbe.org/1ujl PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1ujl RCSB], [https://www.ebi.ac.uk/pdbsum/1ujl PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1ujl ProSAT]</span></td></tr>
+</table>
+== Disease ==
+[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 ==
+[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.
+== Evolutionary Conservation ==
+[[Image:Consurf_key_small.gif|200px|right]]
+Check<jmol>
+  <jmolCheckbox>
+    <scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/uj/1ujl_consurf.spt"</scriptWhenChecked>
+    <scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
+    <text>to colour the structure by Evolutionary Conservation</text>
+  </jmolCheckbox>
+</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=1ujl ConSurf].
+<div style="clear:both"></div>
+<div style="background-color:#fffaf0;">
+== Publication Abstract from PubMed ==
 The HERG K+ channel has very unusual kinetic behaviour that includes slow activation but rapid inactivation. These features are critical for normal cardiac repolarisation as well as in preventing lethal ventricular arrhythmias. Extensive mutagenesis of the HERG K+ channel has allowed identification of which regions of the channel are important for the unusual kinetic behaviour of the channel. Furthermore, structural studies on scorpion toxins that potently inhibit HERG are beginning to provide clues as to the structural differences between HERG and other voltage-gated K+ channels.
-==Disease==
+The HERG K+ channel: progress in understanding the molecular basis of its unusual gating kinetics.,Vandenberg JI, Torres AM, Campbell TJ, Kuchel PW Eur Biophys J. 2004 Apr;33(2):89-97. Epub 2003 Sep 10. PMID:13680209<ref>PMID:13680209</ref>
-Known diseases associated with this structure: Lathosterolosis OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=602286 602286]], Long QT syndrome, acquired, susceptibility to OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=152427 152427]], Long QT syndrome-2 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=152427 152427]], Short QT syndrome-1 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=152427 152427]]
-==About this Structure==
+From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-UJL is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of sequence from [http://en.wikipedia.org/wiki/ ]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=1UJL OCA].
+</div>
+<div class="pdbe-citations 1ujl" style="background-color:#fffaf0;"></div>
-==Reference==
+==See Also==
-The HERG K+ channel: progress in understanding the molecular basis of its unusual gating kinetics., Vandenberg JI, Torres AM, Campbell TJ, Kuchel PW, Eur Biophys J. 2004 Apr;33(2):89-97. Epub 2003 Sep 10. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=13680209 13680209]
+*[[Potassium channel 3D structures|Potassium channel 3D structures]]
-[[Category: Single protein]]
+== References ==
-[[Category: Alewood, P F.]]
+<references/>
-[[Category: Bansal, P S.]]
+__TOC__
-[[Category: Bauskin, A.]]
+</StructureSection>
-[[Category: Breit, S N.]]
+[[Category: Homo sapiens]]
-[[Category: Bursill, J A.]]
+[[Category: Large Structures]]
-[[Category: Campbell, T J.]]
+[[Category: Alewood PF]]
-[[Category: Clarke, C E.]]
+[[Category: Bansal PS]]
-[[Category: Kuchel, P W.]]
+[[Category: Bauskin A]]
-[[Category: Smith, D J.]]
+[[Category: Breit SN]]
-[[Category: Sunde, M.]]
+[[Category: Bursill JA]]
-[[Category: Torres, A M.]]
+[[Category: Campbell TJ]]
-[[Category: Vandenberg, J I.]]
+[[Category: Clarke CE]]
-[[Category: amphiphatic helix]]
+[[Category: Kuchel PW]]
-[[Category: two helices]]
+[[Category: Smith DJ]]
+[[Category: Sunde M]]
-''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 15:25:10 2008''
+[[Category: Torres AM]]
+[[Category: Vandenberg JI]]