3d2s

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[[Image:3d2s.png|left|200px]]
 
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{{STRUCTURE_3d2s| PDB=3d2s | SCENE= }}
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==Crystal structure of MBNL1 tandem zinc finger 3 and 4 domain in complex with CGCUGU RNA==
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<StructureSection load='3d2s' size='340' side='right'caption='[[3d2s]], [[Resolution|resolution]] 1.70&Aring;' scene=''>
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===Crystal structure of MBNL1 tandem zinc finger 3 and 4 domain in complex with CGCUGU RNA===
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== Structural highlights ==
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<table><tr><td colspan='2'>[[3d2s]] 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=3D2S OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=3D2S FirstGlance]. <br>
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{{ABSTRACT_PUBMED_19043415}}
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">X-ray diffraction, [[Resolution|Resolution]] 1.7&#8491;</td></tr>
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<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>
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==About this Structure==
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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=3d2s FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=3d2s OCA], [https://pdbe.org/3d2s PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=3d2s RCSB], [https://www.ebi.ac.uk/pdbsum/3d2s PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=3d2s ProSAT]</span></td></tr>
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[[3d2s]] is a 8 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=3D2S OCA].
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</table>
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== Disease ==
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==Reference==
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[https://www.uniprot.org/uniprot/MBNL1_HUMAN MBNL1_HUMAN] Plays a role in the pathogenesis of dystrophia myotonica type 1 (DM1) [MIM:[https://omim.org/entry/160900 160900]. A muscular disorder characterized by myotonia, muscle wasting in the distal extremities, cataract, hypogonadism, defective endocrine functions, male baldness and cardiac arrhythmias. Note=In muscle cells from DM1 patients, MBNL1 is sequestered by DMPK RNAs containing CUG triplet repeat expansions. MBNL1 binding is proportional to repeat length consistent with the direct correlation between the length of repeat expansion and disease severity.
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<ref group="xtra">PMID:019043415</ref><references group="xtra"/>
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== Function ==
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[https://www.uniprot.org/uniprot/MBNL1_HUMAN MBNL1_HUMAN] Mediates pre-mRNA alternative splicing regulation. Acts either as activator or repressor of splicing on specific pre-mRNA targets. Inhibits cardiac troponin-T (TNNT2) pre-mRNA exon inclusion but induces insulin receptor (IR) pre-mRNA exon inclusion in muscle. Antagonizes the alternative splicing activity pattern of CELF proteins. Regulates the TNNT2 exon 5 skipping through competition with U2AF2. Inhibits the formation of the spliceosome A complex on intron 4 of TNNT2 pre-mRNA. Binds to the stem-loop structure within the polypyrimidine tract of TNNT2 intron 4 during spliceosome assembly. Binds to the 5'-YGCU(U/G)Y-3'consensus sequence. Binds to the IR RNA. Binds to expanded CUG repeat RNA, which folds into a hairpin structure containing GC base pairs and bulged, unpaired U residues.<ref>PMID:10970838</ref> <ref>PMID:15257297</ref> <ref>PMID:16946708</ref> <ref>PMID:18335541</ref> <ref>PMID:19470458</ref>
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== Evolutionary Conservation ==
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[[Image:Consurf_key_small.gif|200px|right]]
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Check<jmol>
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<jmolCheckbox>
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<scriptWhenChecked>; select protein; define ~consurf_to_do selected; consurf_initial_scene = true; script "/wiki/ConSurf/d2/3d2s_consurf.spt"</scriptWhenChecked>
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<scriptWhenUnchecked>script /wiki/extensions/Proteopedia/spt/initialview01.spt</scriptWhenUnchecked>
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<text>to colour the structure by Evolutionary Conservation</text>
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</jmolCheckbox>
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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=3d2s ConSurf].
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<div style="clear:both"></div>
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== References ==
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<references/>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Patel, D J.]]
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[[Category: Large Structures]]
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[[Category: Teplova, M.]]
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[[Category: Patel DJ]]
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[[Category: Metal binding protein-rna complex]]
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[[Category: Teplova M]]
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[[Category: Metal-binding]]
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[[Category: Nucleus]]
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[[Category: Rna]]
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[[Category: Rna-binding]]
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[[Category: Tandem zinc finger domain]]
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[[Category: Zinc-finger]]
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Current revision

Crystal structure of MBNL1 tandem zinc finger 3 and 4 domain in complex with CGCUGU RNA

PDB ID 3d2s

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