View source for 8jpr


==Cryo-EM structure of Y553C human ClC-6==
<StructureSection load='8jpr' size='340' side='right'caption='[[8jpr]], [[Resolution|resolution]] 3.40&Aring;' scene=''>
== Structural highlights ==
<table><tr><td colspan='2'>[[8jpr]] is a 2 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=8JPR OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8JPR FirstGlance]. <br>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 3.4&#8491;</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ATP:ADENOSINE-5-TRIPHOSPHATE'>ATP</scene>, <scene name='pdbligand=CL:CHLORIDE+ION'>CL</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></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=8jpr FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8jpr OCA], [https://pdbe.org/8jpr PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8jpr RCSB], [https://www.ebi.ac.uk/pdbsum/8jpr PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8jpr ProSAT]</span></td></tr>
</table>
== Disease ==
[https://www.uniprot.org/uniprot/CLCN6_HUMAN CLCN6_HUMAN] CLCN6-related childhood-onset progressive neurodegeneration-peripheral neuropathy syndrome. The disease is caused by variants affecting the gene represented in this entry.
== Function ==
[https://www.uniprot.org/uniprot/CLCN6_HUMAN CLCN6_HUMAN] Voltage-gated channel mediating the exchange of chloride ions against protons. Functions as antiporter and contributes to the acidification of the late endosome lumen. The CLC channel family contains both chloride channels and proton-coupled anion transporters that exchange chloride or another anion for protons. The presence of conserved gating glutamate residues is typical for family members that function as antiporters.<ref>PMID:20466723</ref> 
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Zhang SS]]