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3lrq

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{{STRUCTURE_3lrq| PDB=3lrq | SCENE= }}
 
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===Crystal structure of the U-box domain of human ubiquitin-protein ligase (E3), NORTHEAST STRUCTURAL GENOMICS CONSORTIUM TARGET HR4604D.===
 
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==Disease==
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==Crystal structure of the U-box domain of human ubiquitin-protein ligase (E3), NORTHEAST STRUCTURAL GENOMICS CONSORTIUM TARGET HR4604D.==
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[[http://www.uniprot.org/uniprot/TRI37_HUMAN TRI37_HUMAN]] Defects in TRIM37 are the cause of mulibrey nanism (MUL) [MIM:[http://omim.org/entry/253250 253250]]; also known as muscle-liver-brain-eye nanism. MUL is an autosomal recessive disorder that involves several tissues of mesodermal origin, implying a defect in a highly pleiotropic gene. Characteristic features include severe growth failure of prenatal onset and constrictive pericardium with consequent hepatomegaly. In addition, muscle hypotonia, J-shaped sella turcica, yellowish dots in the ocular fundi, typical dysmorphic features and hypoplasia of various endocrine glands causing hormonal deficiency are common.<ref>PMID:15885686</ref><ref>PMID:10888877</ref><ref>PMID:12754710</ref><ref>PMID:15108285</ref><ref>PMID:17100991</ref>
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<StructureSection load='3lrq' size='340' side='right'caption='[[3lrq]], [[Resolution|resolution]] 2.29&Aring;' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[3lrq]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3LRQ OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=3LRQ FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/TRI37_HUMAN TRI37_HUMAN]] E3 ubiquitin-protein ligase.<ref>PMID:15885686</ref>
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</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
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<tr id='NonStdRes'><td class="sblockLbl"><b>[[Non-Standard_Residue|NonStd Res:]]</b></td><td class="sblockDat"><scene name='pdbligand=MSE:SELENOMETHIONINE'>MSE</scene></td></tr>
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==About this Structure==
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<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">KIAA0898, MUL, POB1, TRIM37 ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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[[3lrq]] is a 4 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=3LRQ OCA].
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<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=3lrq FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3lrq OCA], [http://pdbe.org/3lrq PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=3lrq RCSB], [http://www.ebi.ac.uk/pdbsum/3lrq PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=3lrq ProSAT]</span></td></tr>
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</table>
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==Reference==
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== Disease ==
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<references group="xtra"/><references/>
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[[http://www.uniprot.org/uniprot/TRI37_HUMAN TRI37_HUMAN]] Defects in TRIM37 are the cause of mulibrey nanism (MUL) [MIM:[http://omim.org/entry/253250 253250]]; also known as muscle-liver-brain-eye nanism. MUL is an autosomal recessive disorder that involves several tissues of mesodermal origin, implying a defect in a highly pleiotropic gene. Characteristic features include severe growth failure of prenatal onset and constrictive pericardium with consequent hepatomegaly. In addition, muscle hypotonia, J-shaped sella turcica, yellowish dots in the ocular fundi, typical dysmorphic features and hypoplasia of various endocrine glands causing hormonal deficiency are common.<ref>PMID:15885686</ref> <ref>PMID:10888877</ref> <ref>PMID:12754710</ref> <ref>PMID:15108285</ref> <ref>PMID:17100991</ref>
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[[Category: Homo sapiens]]
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== Function ==
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[[Category: Acton, T B.]]
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[[http://www.uniprot.org/uniprot/TRI37_HUMAN TRI37_HUMAN]] E3 ubiquitin-protein ligase.<ref>PMID:15885686</ref>
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[[Category: Chen, Y.]]
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== Evolutionary Conservation ==
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[[Category: Ciccosanti, C.]]
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[[Image:Consurf_key_small.gif|200px|right]]
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[[Category: Everett, J K.]]
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Check<jmol>
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[[Category: Hunt, J F.]]
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<jmolCheckbox>
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[[Category: Kuzin, A.]]
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/lr/3lrq_consurf.spt"</scriptWhenChecked>
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[[Category: Mao, M.]]
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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[[Category: Montelione, G T.]]
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<text>to colour the structure by Evolutionary Conservation</text>
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[[Category: NESG, Northeast Structural Genomics Consortium.]]
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</jmolCheckbox>
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[[Category: Nair, R.]]
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</jmol>, as determined by [http://consurfdb.tau.ac.il/ ConSurfDB]. You may read the [[Conservation%2C_Evolutionary|explanation]] of the method and the full data available from [http://bental.tau.ac.il/new_ConSurfDB/main_output.php?pdb_ID=3lrq ConSurf].
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[[Category: Rost, B.]]
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<div style="clear:both"></div>
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[[Category: Seetharaman, J.]]
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== References ==
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[[Category: Shastry, R.]]
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<references/>
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[[Category: Tong, L.]]
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__TOC__
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[[Category: Xiao, R.]]
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</StructureSection>
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[[Category: Human]]
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[[Category: Large Structures]]
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[[Category: Acton, T B]]
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[[Category: Chen, Y]]
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[[Category: Ciccosanti, C]]
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[[Category: Everett, J K]]
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[[Category: Hunt, J F]]
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[[Category: Kuzin, A]]
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[[Category: Mao, M]]
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[[Category: Montelione, G T]]
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[[Category: Structural genomic]]
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[[Category: Nair, R]]
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[[Category: Rost, B]]
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[[Category: Seetharaman, J]]
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[[Category: Shastry, R]]
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[[Category: Tong, L]]
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[[Category: Xiao, R]]
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[[Category: Coiled coil]]
[[Category: Ligase]]
[[Category: Ligase]]
[[Category: Metal-binding]]
[[Category: Metal-binding]]
[[Category: Nesg]]
[[Category: Nesg]]
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[[Category: Northeast structural genomics consortium]]
 
[[Category: Peroxisome]]
[[Category: Peroxisome]]
[[Category: Phosphoprotein]]
[[Category: Phosphoprotein]]
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[[Category: Protein structure initiative]]
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[[Category: Polymorphism]]
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[[Category: Psi-2]]
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[[Category: PSI, Protein structure initiative]]
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[[Category: Structural genomic]]
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[[Category: Ubl conjugation]]
[[Category: Ubl conjugation pathway]]
[[Category: Ubl conjugation pathway]]
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[[Category: Zinc]]
[[Category: Zinc-finger]]
[[Category: Zinc-finger]]

Current revision

Crystal structure of the U-box domain of human ubiquitin-protein ligase (E3), NORTHEAST STRUCTURAL GENOMICS CONSORTIUM TARGET HR4604D.

PDB ID 3lrq

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