8w4j

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'''Unreleased structure'''
 
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The entry 8w4j is ON HOLD
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==Cryo-EM structure of the KLHL22 E3 ligase bound to human glutamate dehydrogenase I==
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<StructureSection load='8w4j' size='340' side='right'caption='[[8w4j]], [[Resolution|resolution]] 3.06&Aring;' scene=''>
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Authors:
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== Structural highlights ==
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<table><tr><td colspan='2'>[[8w4j]] is a 8 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=8W4J OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=8W4J FirstGlance]. <br>
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Description:
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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]] 3.06&#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=8w4j FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=8w4j OCA], [https://pdbe.org/8w4j PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=8w4j RCSB], [https://www.ebi.ac.uk/pdbsum/8w4j PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=8w4j ProSAT]</span></td></tr>
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/KLH22_HUMAN KLH22_HUMAN] Defects in KLHL22 has been found in a patient with isolated coloboma, a defect of the eye characterized by the absence of ocular structures due to abnormal morphogenesis of the optic cup and stalk, and the fusion of the fetal fissure (optic fissure). Isolated colobomas may be associated with an abnormally small eye (microphthalmia) or small cornea.<ref>PMID:28493397</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/KLH22_HUMAN KLH22_HUMAN] Substrate-specific adapter of a BCR (BTB-CUL3-RBX1) E3 ubiquitin ligase complex required for chromosome alignment and localization of PLK1 at kinetochores. The BCR(KLHL22) ubiquitin ligase complex mediates monoubiquitination of PLK1, leading to PLK1 dissociation from phosphoreceptor proteins and subsequent removal from kinetochores, allowing silencing of the spindle assembly checkpoint (SAC) and chromosome segregation. Monoubiquitination of PLK1 does not lead to PLK1 degradation (PubMed:19995937, PubMed:23455478). The BCR(KLHL22) ubiquitin ligase complex is also responsible for the amino acid-stimulated 'Lys-48' polyubiquitination and proteasomal degradation of DEPDC5. Through the degradation of DEPDC5, releases the GATOR1 complex-mediated inhibition of the TORC1 pathway. It is therefore an amino acid-dependent activator within the amino acid-sensing branch of the TORC1 pathway, indirectly regulating different cellular processes including cell growth and autophagy (PubMed:29769719).<ref>PMID:19995937</ref> <ref>PMID:23455478</ref> <ref>PMID:29769719</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: Su M-Y]]

Current revision

Cryo-EM structure of the KLHL22 E3 ligase bound to human glutamate dehydrogenase I

PDB ID 8w4j

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