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9hge

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Current revision (19:14, 4 December 2025) (edit) (undo)
 
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'''Unreleased structure'''
 
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The entry 9hge is ON HOLD until Paper Publication
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==PB1 domain of p62/SQSTM1==
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<StructureSection load='9hge' size='340' side='right'caption='[[9hge]], [[Resolution|resolution]] 4.50&Aring;' scene=''>
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Authors: Berkamp, S., Jungbluth, L., Katranidis, A., Mostafavi, S., Sachse, C.
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== Structural highlights ==
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<table><tr><td colspan='2'>[[9hge]] is a 40 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=9HGE OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=9HGE FirstGlance]. <br>
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Description: PB1 domain of p62/SQSTM1
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Electron Microscopy, [[Resolution|Resolution]] 4.5&#8491;</td></tr>
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[[Category: Unreleased Structures]]
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<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[https://proteopedia.org/fgij/fg.htm?mol=9hge FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=9hge OCA], [https://pdbe.org/9hge PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=9hge RCSB], [https://www.ebi.ac.uk/pdbsum/9hge PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=9hge ProSAT]</span></td></tr>
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[[Category: Sachse, C]]
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</table>
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[[Category: Berkamp, S]]
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== Disease ==
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[[Category: Katranidis, A]]
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[https://www.uniprot.org/uniprot/SQSTM_HUMAN SQSTM_HUMAN] Defects in SQSTM1 are a cause of Paget disease of bone (PDB) [MIM:[https://omim.org/entry/602080 602080]. PDB is a metabolic bone disease affecting the axial skeleton and characterized by focal areas of increased and disorganized bone turn-over due to activated osteoclasts. Manifestations of the disease include bone pain, deformity, pathological fractures, deafness, neurological complications and increased risk of osteosarcoma. PDB is a chronic disease affecting 2 to 3% of the population above the age of 40 years.<ref>PMID:19931284</ref> <ref>PMID:11992264</ref> <ref>PMID:12374763</ref> <ref>PMID:14584883</ref> <ref>PMID:15146436</ref> <ref>PMID:15207768</ref> <ref>PMID:15125799</ref> <ref>PMID:15176995</ref> Note=In a cell model for Huntington disease (HD), appears to form a shell surrounding aggregates of mutant HTT that may protect cells from apoptosis, possibly by recruiting autophagosomal components to the polyubiquitinylated protein aggregates.<ref>PMID:16286508</ref>
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[[Category: Jungbluth, L]]
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== Function ==
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[[Category: Mostafavi, S]]
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[https://www.uniprot.org/uniprot/SQSTM_HUMAN SQSTM_HUMAN] Required both for the formation and autophagic degradation of polyubiquitin-containing bodies, called ALIS (aggresome-like induced structures). Links ALIS to the autophagic machinery via direct interaction with MAP1 LC3 family members. May regulate the activation of NFKB1 by TNF-alpha, nerve growth factor (NGF) and interleukin-1. May play a role in titin/TTN downstream signaling in muscle cells. May regulate signaling cascades through ubiquitination. Adapter that mediates the interaction between TRAF6 and CYLD (By similarity). May be involved in cell differentiation, apoptosis, immune response and regulation of K(+) channels.<ref>PMID:10356400</ref> <ref>PMID:10747026</ref> <ref>PMID:11244088</ref> <ref>PMID:12471037</ref> <ref>PMID:15340068</ref> <ref>PMID:16079148</ref> <ref>PMID:16286508</ref> <ref>PMID:15953362</ref> <ref>PMID:15911346</ref> <ref>PMID:15802564</ref> <ref>PMID:19931284</ref>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
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[[Category: Homo sapiens]]
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[[Category: Large Structures]]
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[[Category: Berkamp S]]
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[[Category: Jungbluth L]]
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[[Category: Katranidis A]]
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[[Category: Mostafavi S]]
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[[Category: Sachse C]]

Current revision

PB1 domain of p62/SQSTM1

PDB ID 9hge

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