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==Crystal structure of the N-terminal colied-coil domain of human DNA excision repair protein ERCC-6== | ==Crystal structure of the N-terminal colied-coil domain of human DNA excision repair protein ERCC-6== | ||
<StructureSection load='4cvo' size='340' side='right' caption='[[4cvo]], [[Resolution|resolution]] 1.85Å' scene=''> | <StructureSection load='4cvo' size='340' side='right'caption='[[4cvo]], [[Resolution|resolution]] 1.85Å' scene=''> | ||
== Structural highlights == | == Structural highlights == | ||
<table><tr><td colspan='2'>[[4cvo]] is a 1 chain structure with sequence from [ | <table><tr><td colspan='2'>[[4cvo]] is a 1 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=4CVO OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=4CVO FirstGlance]. <br> | ||
</td></tr><tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat"><scene name='pdbligand=MG:MAGNESIUM+ION'>MG</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]] 1.85Å</td></tr> | ||
<tr id='resources'><td class="sblockLbl"><b>Resources:</b></td><td class="sblockDat"><span class='plainlinks'>[ | <tr id='ligand'><td class="sblockLbl"><b>[[Ligand|Ligands:]]</b></td><td class="sblockDat" id="ligandDat"><scene name='pdbligand=MG:MAGNESIUM+ION'>MG</scene></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=4cvo FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=4cvo OCA], [https://pdbe.org/4cvo PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=4cvo RCSB], [https://www.ebi.ac.uk/pdbsum/4cvo PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=4cvo ProSAT]</span></td></tr> | |||
</table> | </table> | ||
== Disease == | == Disease == | ||
[ | [https://www.uniprot.org/uniprot/ERCC6_HUMAN ERCC6_HUMAN] Cockayne syndrome type 1;Cockayne syndrome type 3;Cockayne syndrome type 2;UV-sensitive syndrome;Age-related macular degeneration;COFS syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. Disease susceptibility is associated with variations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. | ||
== Function == | == Function == | ||
[ | [https://www.uniprot.org/uniprot/ERCC6_HUMAN ERCC6_HUMAN] Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the at sites of RNA polymerase II-blocking lesions.<ref>PMID:15548521</ref> <ref>PMID:16916636</ref> <ref>PMID:20541997</ref> | ||
== References == | == References == | ||
<references/> | <references/> | ||
__TOC__ | __TOC__ | ||
</StructureSection> | </StructureSection> | ||
[[Category: | [[Category: Homo sapiens]] | ||
[[Category: | [[Category: Large Structures]] | ||
[[Category: | [[Category: Arrowsmith CH]] | ||
[[Category: | [[Category: Bountra C]] | ||
[[Category: Edwards | [[Category: Edwards A]] | ||
[[Category: Gileadi | [[Category: Gileadi O]] | ||
[[Category: Krojer | [[Category: Krojer T]] | ||
[[Category: Newman | [[Category: Newman JA]] | ||
[[Category: | [[Category: Von Delft F]] | ||
Latest revision as of 11:24, 23 October 2024
Crystal structure of the N-terminal colied-coil domain of human DNA excision repair protein ERCC-6Crystal structure of the N-terminal colied-coil domain of human DNA excision repair protein ERCC-6
Structural highlights
DiseaseERCC6_HUMAN Cockayne syndrome type 1;Cockayne syndrome type 3;Cockayne syndrome type 2;UV-sensitive syndrome;Age-related macular degeneration;COFS syndrome. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. Disease susceptibility is associated with variations affecting the gene represented in this entry. The disease is caused by mutations affecting the gene represented in this entry. FunctionERCC6_HUMAN Essential factor involved in transcription-coupled nucleotide excision repair which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes. Upon DNA-binding, it locally modifies DNA conformation by wrapping the DNA around itself, thereby modifying the interface between stalled RNA polymerase II and DNA. It is required for transcription-coupled repair complex formation. It recruits the CSA complex (DCX(ERCC8) complex), nucleotide excision repair proteins and EP300 to the at sites of RNA polymerase II-blocking lesions.[1] [2] [3] References
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