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5ofa

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Crystal structure of human MORC2 (residues 1-603) with spinal muscular atrophy mutation T424R

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Categories: Homo sapiens | Large Structures | Douse CH | Liu Y | Modis Y

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 ==Crystal structure of human MORC2 (residues 1-603) with spinal muscular atrophy mutation T424R==
-<StructureSection load='5ofa' size='340' side='right' caption='[[5ofa]], [[Resolution|resolution]] 2.57&Aring;' scene=''>
+<StructureSection load='5ofa' size='340' side='right'caption='[[5ofa]], [[Resolution|resolution]] 2.57&Aring;' scene=''>
 == Structural highlights ==
-<table><tr><td colspan='2'>[[5ofa]] is a 2 chain structure with sequence from [http://en.wikipedia.org/wiki/Human Human]. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=5OFA OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5OFA FirstGlance]. <br>
+<table><tr><td colspan='2'>[[5ofa]] is a 2 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=5OFA OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=5OFA FirstGlance]. <br>
-</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=ANP:PHOSPHOAMINOPHOSPHONIC+ACID-ADENYLATE+ESTER'>ANP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <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">X-ray diffraction, [[Resolution|Resolution]] 2.57&#8491;</td></tr>
-<tr id='gene'><td class="sblockLbl"><b>[[Gene|Gene:]]</b></td><td class="sblockDat">MORC2, KIAA0852, ZCWCC1 ([http://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=ANP:PHOSPHOAMINOPHOSPHONIC+ACID-ADENYLATE+ESTER'>ANP</scene>, <scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene>, <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'>[http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=5ofa FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5ofa OCA], [http://pdbe.org/5ofa PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=5ofa RCSB], [http://www.ebi.ac.uk/pdbsum/5ofa PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=5ofa 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=5ofa FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=5ofa OCA], [https://pdbe.org/5ofa PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=5ofa RCSB], [https://www.ebi.ac.uk/pdbsum/5ofa PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=5ofa ProSAT]</span></td></tr>
 </table>
 == Disease ==
-[[http://www.uniprot.org/uniprot/MORC2_HUMAN MORC2_HUMAN]] The disease is caused by mutations affecting the gene represented in this entry.
+[https://www.uniprot.org/uniprot/MORC2_HUMAN MORC2_HUMAN] The disease is caused by mutations affecting the gene represented in this entry.
 == Function ==
-[[http://www.uniprot.org/uniprot/MORC2_HUMAN MORC2_HUMAN]] Exhibits a cytosolic function in lipogenesis, adipogenic differentiation, and lipid homeostasis by increasing the activity of ACLY, possibly preventing its dephosphorylation (PubMed:24286864). May act as a transcriptional repressor (PubMed:20225202). Down-regulates CA9 expression (PubMed:20110259).<ref>PMID:20110259</ref> <ref>PMID:20225202</ref> <ref>PMID:24286864</ref>
+[https://www.uniprot.org/uniprot/MORC2_HUMAN MORC2_HUMAN] Exhibits a cytosolic function in lipogenesis, adipogenic differentiation, and lipid homeostasis by increasing the activity of ACLY, possibly preventing its dephosphorylation (PubMed:24286864). May act as a transcriptional repressor (PubMed:20225202). Down-regulates CA9 expression (PubMed:20110259).<ref>PMID:20110259</ref> <ref>PMID:20225202</ref> <ref>PMID:24286864</ref>
-<div style="background-color:#fffaf0;">
-== Publication Abstract from PubMed ==
-Missense mutations in MORC2 cause neuropathies including spinal muscular atrophy and Charcot-Marie-Tooth disease. We recently identified MORC2 as an effector of epigenetic silencing by the human silencing hub (HUSH). Here we report the biochemical and cellular activities of MORC2 variants, alongside crystal structures of wild-type and neuropathic forms of a human MORC2 fragment comprising the GHKL-type ATPase module and CW-type zinc finger. This fragment dimerizes upon binding ATP and contains a hinged, functionally critical coiled-coil insertion absent in other GHKL ATPases. We find that dimerization and DNA binding of the MORC2 ATPase module transduce HUSH-dependent silencing. Disease mutations change the dynamics of dimerization by distinct structural mechanisms: destabilizing the ATPase-CW module, trapping the ATP lid, or perturbing the dimer interface. These defects lead to the modulation of HUSH function, thus providing a molecular basis for understanding MORC2-associated neuropathies.
-Neuropathic MORC2 mutations perturb GHKL ATPase dimerization dynamics and epigenetic silencing by multiple structural mechanisms.,Douse CH, Bloor S, Liu Y, Shamin M, Tchasovnikarova IA, Timms RT, Lehner PJ, Modis Y Nat Commun. 2018 Feb 13;9(1):651. doi: 10.1038/s41467-018-03045-x. PMID:29440755<ref>PMID:29440755</ref>
-From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
-</div>
-<div class="pdbe-citations 5ofa" style="background-color:#fffaf0;"></div>
 == References ==
 <references/>
 __TOC__
 </StructureSection>
-[[Category: Human]]
+[[Category: Homo sapiens]]
-[[Category: Douse, C H]]
+[[Category: Large Structures]]
-[[Category: Liu, Y]]
+[[Category: Douse CH]]
-[[Category: Modis, Y]]
+[[Category: Liu Y]]
-[[Category: Charcot-marie-tooth disease]]
+[[Category: Modis Y]]
-[[Category: Chromatin remodeler]]
-[[Category: Coiled-coil]]
-[[Category: Cw domain]]
-[[Category: Dna binding protein]]
-[[Category: Epigenetic silencing]]
-[[Category: Ghkl atpase]]
-[[Category: Nuclear protein]]
-[[Category: Spinal muscular atrophy]]
-[[Category: Transcriptional repressor]]

5ofa

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Crystal structure of human MORC2 (residues 1-603) with spinal muscular atrophy mutation T424R

Structural highlights

Disease

Function

References

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