6u3r
From Proteopedia
(Difference between revisions)
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| - | '''Unreleased structure''' | ||
| - | The entry | + | ==Solution NMR structure of the DNAJB6b deltaST variant (Aligned on the J domain)== |
| - | + | <StructureSection load='6u3r' size='340' side='right'caption='[[6u3r]], [[NMR_Ensembles_of_Models | 10 NMR models]]' scene=''> | |
| - | + | == Structural highlights == | |
| - | + | <table><tr><td colspan='2'>[[6u3r]] is a 1 chain structure. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6U3R OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6U3R FirstGlance]. <br> | |
| - | + | </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=6u3r FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6u3r OCA], [http://pdbe.org/6u3r PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6u3r RCSB], [http://www.ebi.ac.uk/pdbsum/6u3r PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6u3r ProSAT]</span></td></tr> | |
| - | [[Category: | + | </table> |
| + | == Disease == | ||
| + | [[http://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 == | ||
| + | [[http://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> | ||
| + | == References == | ||
| + | <references/> | ||
| + | __TOC__ | ||
| + | </StructureSection> | ||
| + | [[Category: Large Structures]] | ||
| + | [[Category: Clore, G M]] | ||
| + | [[Category: Karamanos, T K]] | ||
| + | [[Category: Amyloid]] | ||
| + | [[Category: Anti-aggregation]] | ||
| + | [[Category: Chaperone]] | ||
| + | [[Category: Hsp40]] | ||
Revision as of 05:28, 10 October 2019
Solution NMR structure of the DNAJB6b deltaST variant (Aligned on the J domain)
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