7qby
From Proteopedia
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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 | + | <StructureSection load='7qby' size='340' side='right'caption='[[7qby]]' scene=''> |
== Structural highlights == | == Structural highlights == | ||
| - | <table><tr><td colspan='2'> | + | <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=' | + | </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> | ||
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| + | ==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 | + | [[Category: Cawood EE]] |
| - | [[Category: Karamanos | + | [[Category: Karamanos TK]] |
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Current revision
Refined structure of the T193A mutant in the C-terminal domain of DNAJB6b
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