2dix

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{{STRUCTURE_2dix| PDB=2dix | SCENE= }}
 
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===Solution structure of the DSRM domain of Protein activator of the interferon-induced protein kinase===
 
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==Disease==
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==Solution structure of the DSRM domain of Protein activator of the interferon-induced protein kinase==
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[[http://www.uniprot.org/uniprot/PRKRA_HUMAN PRKRA_HUMAN]] Defects in PRKRA are the cause of dystonia type 16 (DYT16) [MIM:[http://omim.org/entry/612067 612067]]. DYT16 is an early-onset dystonia-parkinsonism disorder. Dystonia is defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYT16 patients have progressive, generalized dystonia with axial muscle involvement, oro-mandibular (sardonic smile) and laryngeal dystonia and, in some cases, parkinsonian features.<ref>PMID:18243799</ref><ref>PMID:18420150</ref>
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<StructureSection load='2dix' size='340' side='right'caption='[[2dix]]' scene=''>
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== Structural highlights ==
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==Function==
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<table><tr><td colspan='2'>[[2dix]] 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=2DIX OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2DIX FirstGlance]. <br>
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[[http://www.uniprot.org/uniprot/PRKRA_HUMAN PRKRA_HUMAN]] Activates EIF2AK2/PKR in the absence of double stranded RNA (dsRNA), leading to phosphorylation of EIF2S1/EFI2-alpha and inhibition of translation and induction of apoptosis. Required for siRNA production by DICER1 and for subsequent siRNA-mediated post-transcriptional gene silencing. Does not seem to be required for processing of pre-miRNA to miRNA by DICER1.<ref>PMID:9687506</ref><ref>PMID:10336432</ref><ref>PMID:11238927</ref><ref>PMID:16424907</ref><ref>PMID:16982605</ref><ref>PMID:17452327</ref>
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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=2dix FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dix OCA], [https://pdbe.org/2dix PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2dix RCSB], [https://www.ebi.ac.uk/pdbsum/2dix PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2dix ProSAT], [https://www.topsan.org/Proteins/RSGI/2dix TOPSAN]</span></td></tr>
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==About this Structure==
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</table>
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[[2dix]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Homo_sapiens Homo sapiens]. Full experimental information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=2DIX OCA].
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== Disease ==
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[https://www.uniprot.org/uniprot/PRKRA_HUMAN PRKRA_HUMAN] Defects in PRKRA are the cause of dystonia type 16 (DYT16) [MIM:[https://omim.org/entry/612067 612067]. DYT16 is an early-onset dystonia-parkinsonism disorder. Dystonia is defined by the presence of sustained involuntary muscle contraction, often leading to abnormal postures. DYT16 patients have progressive, generalized dystonia with axial muscle involvement, oro-mandibular (sardonic smile) and laryngeal dystonia and, in some cases, parkinsonian features.<ref>PMID:18243799</ref> <ref>PMID:18420150</ref>
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==Reference==
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== Function ==
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<references group="xtra"/><references/>
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[https://www.uniprot.org/uniprot/PRKRA_HUMAN PRKRA_HUMAN] Activates EIF2AK2/PKR in the absence of double stranded RNA (dsRNA), leading to phosphorylation of EIF2S1/EFI2-alpha and inhibition of translation and induction of apoptosis. Required for siRNA production by DICER1 and for subsequent siRNA-mediated post-transcriptional gene silencing. Does not seem to be required for processing of pre-miRNA to miRNA by DICER1.<ref>PMID:9687506</ref> <ref>PMID:10336432</ref> <ref>PMID:11238927</ref> <ref>PMID:16424907</ref> <ref>PMID:16982605</ref> <ref>PMID:17452327</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/di/2dix_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=2dix 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: Dang, W.]]
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[[Category: Large Structures]]
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[[Category: Inoue, M.]]
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[[Category: Dang W]]
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[[Category: Kigawa, T.]]
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[[Category: Inoue M]]
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[[Category: Muto, Y.]]
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[[Category: Kigawa T]]
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[[Category: RSGI, RIKEN Structural Genomics/Proteomics Initiative.]]
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[[Category: Muto Y]]
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[[Category: Shirouzu, M.]]
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[[Category: Shirouzu M]]
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[[Category: Terada, T.]]
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[[Category: Terada T]]
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[[Category: Yokoyama, S.]]
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[[Category: Yokoyama S]]
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[[Category: Dsrm domain]]
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[[Category: Hypothetical protein prkra]]
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[[Category: National project on protein structural and functional analyse]]
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[[Category: Nppsfa]]
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[[Category: Riken structural genomics/proteomics initiative]]
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[[Category: Rna binding protein]]
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[[Category: Rsgi]]
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[[Category: Structural genomic]]
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[[Category: Structure genomic]]
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Current revision

Solution structure of the DSRM domain of Protein activator of the interferon-induced protein kinase

PDB ID 2dix

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OCA

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