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==Crystal Structure of the HHD2 Domain of Whirlin==
==Crystal Structure of the HHD2 Domain of Whirlin==
<StructureSection load='6fdd' size='340' side='right' caption='[[6fdd]], [[Resolution|resolution]] 1.75&Aring;' scene=''>
<StructureSection load='6fdd' size='340' side='right'caption='[[6fdd]], [[Resolution|resolution]] 1.75&Aring;' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[6fdd]] is a 6 chain structure with sequence from [http://en.wikipedia.org/wiki/Lk3_transgenic_mice Lk3 transgenic mice]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6FDD OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6FDD FirstGlance]. <br>
<table><tr><td colspan='2'>[[6fdd]] is a 6 chain structure with sequence from [https://en.wikipedia.org/wiki/Mus_musculus Mus musculus]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6FDD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=6FDD FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.75&#8491;</td></tr>
<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene>, <scene name='pdbligand=SO4:SULFATE+ION'>SO4</scene></td></tr>
<tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[6fde|6fde]]</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=6fdd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6fdd OCA], [https://pdbe.org/6fdd PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=6fdd RCSB], [https://www.ebi.ac.uk/pdbsum/6fdd PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=6fdd ProSAT]</span></td></tr>
<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">Whrn, Dfnb31, Kiaa1526 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=10090 LK3 transgenic mice])</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=6fdd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6fdd OCA], [http://pdbe.org/6fdd PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6fdd RCSB], [http://www.ebi.ac.uk/pdbsum/6fdd PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6fdd ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[http://www.uniprot.org/uniprot/WHRN_MOUSE WHRN_MOUSE]] Defects in Whrn are the cause of the phenotype whirler (wi). Mutants are characterized by deafness due to malformation of the cochlear inner and outer hair cells and by circling behavior. Stereocilia are shorter and wider than in wild-type animals and there is a decrease in the number of actin filaments in inner and outer hair cells. The number of outer hair cell stereocilia is reduced with increased spacing between them.<ref>PMID:12124769</ref> <ref>PMID:12833159</ref> <ref>PMID:17326148</ref>
[https://www.uniprot.org/uniprot/WHRN_MOUSE WHRN_MOUSE] Defects in Whrn are the cause of the phenotype whirler (wi). Mutants are characterized by deafness due to malformation of the cochlear inner and outer hair cells and by circling behavior. Stereocilia are shorter and wider than in wild-type animals and there is a decrease in the number of actin filaments in inner and outer hair cells. The number of outer hair cell stereocilia is reduced with increased spacing between them.<ref>PMID:12124769</ref> <ref>PMID:12833159</ref> <ref>PMID:17326148</ref>  
== Function ==
== Function ==
[[http://www.uniprot.org/uniprot/WHRN_MOUSE WHRN_MOUSE]] Necessary for elongation and maintenance of inner and outer hair cell stereocilia in the organ of Corti in the inner ear.<ref>PMID:15590699</ref>
[https://www.uniprot.org/uniprot/WHRN_MOUSE WHRN_MOUSE] Necessary for elongation and maintenance of inner and outer hair cell stereocilia in the organ of Corti in the inner ear.<ref>PMID:15590699</ref>  
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Lk3 transgenic mice]]
[[Category: Large Structures]]
[[Category: Cordier, F]]
[[Category: Mus musculus]]
[[Category: Delhommel, F]]
[[Category: Cordier F]]
[[Category: Haouz, A]]
[[Category: Delhommel F]]
[[Category: Saul, F]]
[[Category: Haouz A]]
[[Category: Wolff, N]]
[[Category: Saul F]]
[[Category: Deafness]]
[[Category: Wolff N]]
[[Category: Hearing]]
[[Category: Scaffold protein]]
[[Category: Structural protein]]
[[Category: Usher syndrome]]
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