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==Structure of the MID1 tandem B-boxes reveals an interaction reminiscent of intermolecular RING heterodimers==
==Structure of the MID1 tandem B-boxes reveals an interaction reminiscent of intermolecular RING heterodimers==
<StructureSection load='2jun' size='340' side='right'caption='[[2jun]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
<StructureSection load='2jun' size='340' side='right'caption='[[2jun]]' scene=''>
== Structural highlights ==
== Structural highlights ==
<table><tr><td colspan='2'>[[2jun]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Human Human]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2JUN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JUN FirstGlance]. <br>
<table><tr><td colspan='2'>[[2jun]] is a 1 chain structure with sequence from [https://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2JUN OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JUN FirstGlance]. <br>
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></td></tr>
</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='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">MID1, FXY, RNF59, TRIM18, XPRF ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
<tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=ZN:ZINC+ION'>ZN</scene></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=2jun FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jun OCA], [https://pdbe.org/2jun PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jun RCSB], [https://www.ebi.ac.uk/pdbsum/2jun PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jun 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=2jun FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jun OCA], [https://pdbe.org/2jun PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jun RCSB], [https://www.ebi.ac.uk/pdbsum/2jun PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jun ProSAT]</span></td></tr>
</table>
</table>
== Disease ==
== Disease ==
[[https://www.uniprot.org/uniprot/TRI18_HUMAN TRI18_HUMAN]] Defects in MID1 are the cause of Opitz GBBB syndrome 1 (OGS1) [MIM:[https://omim.org/entry/300000 300000]]. A congenital midline malformation syndrome characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. Note=MID1 mutations produce proteins with a decreased affinity for microtubules.<ref>PMID:9354791</ref> <ref>PMID:11030761</ref> <ref>PMID:9718340</ref> <ref>PMID:15558842</ref>
[https://www.uniprot.org/uniprot/TRI18_HUMAN TRI18_HUMAN] Defects in MID1 are the cause of Opitz GBBB syndrome 1 (OGS1) [MIM:[https://omim.org/entry/300000 300000]. A congenital midline malformation syndrome characterized by hypertelorism, genital-urinary defects such as hypospadias in males and splayed labia in females, lip-palate-laryngotracheal clefts, imperforate anus, developmental delay and congenital heart defects. Note=MID1 mutations produce proteins with a decreased affinity for microtubules.<ref>PMID:9354791</ref> <ref>PMID:11030761</ref> <ref>PMID:9718340</ref> <ref>PMID:15558842</ref>  
== Function ==
== Function ==
[[https://www.uniprot.org/uniprot/TRI18_HUMAN TRI18_HUMAN]] Has E3 ubiquitin ligase activity towards IGBP1, promoting its monoubiquitination, which results in deprotection of the catalytic subunit of protein phosphatase PP2A, and its subsequent degradation by polyubiquitination.<ref>PMID:10400985</ref> <ref>PMID:11685209</ref> <ref>PMID:22613722</ref>
[https://www.uniprot.org/uniprot/TRI18_HUMAN TRI18_HUMAN] Has E3 ubiquitin ligase activity towards IGBP1, promoting its monoubiquitination, which results in deprotection of the catalytic subunit of protein phosphatase PP2A, and its subsequent degradation by polyubiquitination.<ref>PMID:10400985</ref> <ref>PMID:11685209</ref> <ref>PMID:22613722</ref>  
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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__TOC__
__TOC__
</StructureSection>
</StructureSection>
[[Category: Human]]
[[Category: Homo sapiens]]
[[Category: Large Structures]]
[[Category: Large Structures]]
[[Category: Cox, T C]]
[[Category: Cox TC]]
[[Category: Jakkidi, M]]
[[Category: Jakkidi M]]
[[Category: Massiah, M A]]
[[Category: Massiah MA]]
[[Category: Short, K M]]
[[Category: Short KM]]
[[Category: Simmons, B N]]
[[Category: Simmons BN]]
[[Category: Singireddy, S]]
[[Category: Singireddy S]]
[[Category: Tao, H]]
[[Category: Tao H]]
[[Category: Alternative splicing]]
[[Category: B-box]]
[[Category: Coiled coil]]
[[Category: Cytoplasm]]
[[Category: Cytoskeleton]]
[[Category: Disease mutation]]
[[Category: Ligase]]
[[Category: Metal-binding]]
[[Category: Microtubule]]
[[Category: Midline 1]]
[[Category: Phosphorylation]]
[[Category: Ring finger]]
[[Category: Trim]]
[[Category: Ubl conjugation pathway]]
[[Category: Zinc]]
[[Category: Zinc-finger]]
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