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2dix

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==Solution structure of the DSRM domain of Protein activator of the interferon-induced protein kinase==
==Solution structure of the DSRM domain of Protein activator of the interferon-induced protein kinase==
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<StructureSection load='2dix' size='340' side='right' caption='[[2dix]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
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<StructureSection load='2dix' size='340' side='right'caption='[[2dix]], [[NMR_Ensembles_of_Models | 20 NMR models]]' scene=''>
== Structural highlights ==
== Structural highlights ==
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<table><tr><td colspan='2'>[[2dix]] is a 1 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. 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 [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=2DIX FirstGlance]. <br>
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<table><tr><td colspan='2'>[[2dix]] 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=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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</td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PRKRA, DKFZp564I0123, PACT ([http://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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</td></tr><tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">PRKRA, DKFZp564I0123, PACT ([https://www.ncbi.nlm.nih.gov/Taxonomy/Browser/wwwtax.cgi?mode=Info&srchmode=5&id=9606 HUMAN])</td></tr>
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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=2dix FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2dix OCA], [http://pdbe.org/2dix PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=2dix RCSB], [http://www.ebi.ac.uk/pdbsum/2dix PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=2dix ProSAT], [http://www.topsan.org/Proteins/RSGI/2dix TOPSAN]</span></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>
</table>
</table>
== Disease ==
== Disease ==
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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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[[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>
== Function ==
== Function ==
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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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[[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>
== Evolutionary Conservation ==
== Evolutionary Conservation ==
[[Image:Consurf_key_small.gif|200px|right]]
[[Image:Consurf_key_small.gif|200px|right]]
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</StructureSection>
</StructureSection>
[[Category: Human]]
[[Category: Human]]
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[[Category: Large Structures]]
[[Category: Dang, W]]
[[Category: Dang, W]]
[[Category: Inoue, M]]
[[Category: Inoue, M]]

Revision as of 12:04, 3 February 2021

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

PDB ID 2dix

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