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==Refined structure of the T193A mutant in the C-terminal domain of DNAJB6b==
==Refined structure of the T193A mutant in the C-terminal domain of DNAJB6b==
<StructureSection load='7qby' size='340' side='right'caption='[[7qby]], [[NMR_Ensembles_of_Models | 10 NMR models]]' scene=''>
<StructureSection load='7qby' size='340' side='right'caption='[[7qby]]' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[7qby]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7QBY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7QBY FirstGlance]. <br>
<table><tr><td colspan='2'>Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=7QBY OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=7QBY FirstGlance]. <br>
</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat"><div style='overflow: auto; max-height: 3em;'>[[6u3s|6u3s]], [[6u3r|6u3r]], [[7jsq|7jsq]]</div></td></tr>
</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=7qby FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7qby OCA], [https://pdbe.org/7qby PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7qby RCSB], [https://www.ebi.ac.uk/pdbsum/7qby PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7qby ProSAT]</span></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=7qby FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=7qby OCA], [https://pdbe.org/7qby PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=7qby RCSB], [https://www.ebi.ac.uk/pdbsum/7qby PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=7qby ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
[[https://www.uniprot.org/uniprot/DNJB6_HUMAN DNJB6_HUMAN]] Autosomal dominant limb-girdle muscular dystrophy type 1D. The disease is caused by mutations affecting the gene represented in this entry. There is evidence that LGMDD1 is caused by dysfunction of isoform B (PubMed:22366786).<ref>PMID:22366786</ref> 
== Function ==
[[https://www.uniprot.org/uniprot/DNJB6_HUMAN DNJB6_HUMAN]] Plays an indispensable role in the organization of KRT8/KRT18 filaments. Acts as an endogenous molecular chaperone for neuronal proteins including huntingtin. Suppresses aggregation and toxicity of polyglutamine-containing, aggregation-prone proteins. Isoform B but not isoform A inhibits huntingtin aggregation. Has a stimulatory effect on the ATPase activity of HSP70 in a dose-dependent and time-dependent manner and hence acts as a co-chaperone of HSP70. Also reduces cellular toxicity and caspase-3 activity.<ref>PMID:10954706</ref> <ref>PMID:11896048</ref> <ref>PMID:20159555</ref> <ref>PMID:22366786</ref> <ref>PMID:28233300</ref> 
<div style="background-color:#fffaf0;">
<div style="background-color:#fffaf0;">
== Publication Abstract from PubMed ==
== Publication Abstract from PubMed ==
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</div>
</div>
<div class="pdbe-citations 7qby" style="background-color:#fffaf0;"></div>
<div class="pdbe-citations 7qby" style="background-color:#fffaf0;"></div>
==See Also==
*[[DnaJ homolog 3D structures|DnaJ homolog 3D structures]]
== References ==
== References ==
<references/>
<references/>
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</StructureSection>
</StructureSection>
[[Category: Large Structures]]
[[Category: Large Structures]]
[[Category: Cawood, E E]]
[[Category: Cawood EE]]
[[Category: Karamanos, T K]]
[[Category: Karamanos TK]]
[[Category: Anti-aggregation]]
[[Category: Chaperone]]
[[Category: Hsp40 chaperone]]
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