1g5v

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[[Image:1g5v.gif|left|200px]]<br /><applet load="1g5v" size="350" color="white" frame="true" align="right" spinBox="true"
 
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caption="1g5v" />
 
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'''SOLUTION STRUCTURE OF THE TUDOR DOMAIN OF THE HUMAN SMN PROTEIN'''<br />
 
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==Overview==
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==SOLUTION STRUCTURE OF THE TUDOR DOMAIN OF THE HUMAN SMN PROTEIN==
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Spinal muscular atrophy (SMA) is a common motor neuron disease that results from mutations in the Survival of Motor Neuron (SMN) gene. The SMN protein plays a crucial role in the assembly of spliceosomal uridine-rich small nuclear ribonucleoprotein (U snRNP) complexes via binding to the spliceosomal Sm core proteins. SMN contains a central Tudor domain that facilitates the SMN-Sm protein interaction. A SMA-causing point mutation (E134K) within the SMN Tudor domain prevents Sm binding. Here, we have determined the three-dimensional structure of the Tudor domain of human SMN. The structure exhibits a conserved negatively charged surface that is shown to interact with the C-terminal Arg and Gly-rich tails of Sm proteins. The E134K mutation does not disrupt the Tudor structure but affects the charge distribution within this binding site. An intriguing structural similarity between the Tudor domain and the Sm proteins suggests the presence of an additional binding interface that resembles that in hetero-oligomeric complexes of Sm proteins. Our data provide a structural basis for a molecular defect underlying SMA.
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<StructureSection load='1g5v' size='340' side='right'caption='[[1g5v]]' scene=''>
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== Structural highlights ==
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==Disease==
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<table><tr><td colspan='2'>[[1g5v]] 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=1G5V OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=1G5V FirstGlance]. <br>
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Known diseases associated with this structure: Spinal muscular atrophy-1 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600354 600354]], Spinal muscular atrophy-2 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600354 600354]], Spinal muscular atrophy-3 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600354 600354]], Spinal muscular atrophy-4 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=600354 600354]]
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</td></tr><tr id='method'><td class="sblockLbl"><b>[[Empirical_models|Method:]]</b></td><td class="sblockDat" id="methodDat">Solution NMR</td></tr>
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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=1g5v FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=1g5v OCA], [https://pdbe.org/1g5v PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=1g5v RCSB], [https://www.ebi.ac.uk/pdbsum/1g5v PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=1g5v ProSAT]</span></td></tr>
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==About this Structure==
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</table>
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1G5V is a [http://en.wikipedia.org/wiki/Single_protein Single protein] structure of 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=1G5V OCA].
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== Function ==
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[https://www.uniprot.org/uniprot/SMN_HUMAN SMN_HUMAN]
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==Reference==
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== Evolutionary Conservation ==
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SMN tudor domain structure and its interaction with the Sm proteins., Selenko P, Sprangers R, Stier G, Buhler D, Fischer U, Sattler M, Nat Struct Biol. 2001 Jan;8(1):27-31. PMID:[http://ispc.weizmann.ac.il//pmbin/getpm?pmid=11135666 11135666]
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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/g5/1g5v_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=1g5v ConSurf].
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<div style="clear:both"></div>
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__TOC__
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</StructureSection>
[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Single protein]]
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[[Category: Large Structures]]
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[[Category: Buehler, D.]]
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[[Category: Buehler D]]
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[[Category: Fischer, U.]]
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[[Category: Fischer U]]
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[[Category: Sattler, M.]]
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[[Category: Sattler M]]
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[[Category: Selenko, P.]]
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[[Category: Selenko P]]
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[[Category: Sprangers, R.]]
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[[Category: Sprangers R]]
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[[Category: Stier, G.]]
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[[Category: Stier G]]
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[[Category: mrna processing]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Thu Feb 21 12:46:24 2008''
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Current revision

SOLUTION STRUCTURE OF THE TUDOR DOMAIN OF THE HUMAN SMN PROTEIN

PDB ID 1g5v

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