2jpd

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{{Seed}}
 
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[[Image:2jpd.png|left|200px]]
 
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==Solution structure of the ERCC1 central domain==
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The line below this paragraph, containing "STRUCTURE_2jpd", creates the "Structure Box" on the page.
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<StructureSection load='2jpd' size='340' side='right'caption='[[2jpd]]' scene=''>
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You may change the PDB parameter (which sets the PDB file loaded into the applet)
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== Structural highlights ==
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or the SCENE parameter (which sets the initial scene displayed when the page is loaded),
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<table><tr><td colspan='2'>[[2jpd]] 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=2JPD OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JPD FirstGlance]. <br>
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or leave the SCENE parameter empty for the default display.
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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=2jpd FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jpd OCA], [https://pdbe.org/2jpd PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jpd RCSB], [https://www.ebi.ac.uk/pdbsum/2jpd PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jpd ProSAT]</span></td></tr>
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{{STRUCTURE_2jpd| PDB=2jpd | SCENE= }}
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</table>
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== Disease ==
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[https://www.uniprot.org/uniprot/ERCC1_HUMAN ERCC1_HUMAN] Defects in ERCC1 are the cause of cerebro-oculo-facio-skeletal syndrome type 4 (COFS4) [MIM:[https://omim.org/entry/610758 610758]. COFS is a degenerative autosomal recessive disorder of prenatal onset affecting the brain, eye and spinal cord. After birth, it leads to brain atrophy, hypoplasia of the corpus callosum, hypotonia, cataracts, microcornea, optic atrophy, progressive joint contractures and growth failure. Facial dysmorphism is a constant feature. Abnormalities of the skull, eyes, limbs, heart and kidney also occur.<ref>PMID:17273966</ref>
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== Function ==
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[https://www.uniprot.org/uniprot/ERCC1_HUMAN ERCC1_HUMAN] Structure-specific DNA repair endonuclease responsible for the 5'-incision during DNA repair.
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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/jp/2jpd_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=2jpd ConSurf].
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<div style="clear:both"></div>
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<div style="background-color:#fffaf0;">
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== Publication Abstract from PubMed ==
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Human ERCC1/XPF is a structure-specific endonuclease involved in multiple DNA repair pathways. We present the solution structure of the non-catalytic ERCC1 central domain. Although this domain shows structural homology with the catalytically active XPF nuclease domain, functional investigation reveals a completely distinct function for the ERCC1 central domain by performing interactions with both XPA and single-stranded DNA. These interactions are non-competitive and can occur simultaneously through distinct interaction surfaces. Interestingly, the XPA binding by ERCC1 and the catalytic function of XPF are dependent on a structurally homologous region of the two proteins. Although these regions are strictly conserved in each protein family, amino acid composition and surface characteristics are distinct. We discuss the possibility that after XPF gene duplication, the redundant ERCC1 central domain acquired novel functions, thereby increasing the fidelity of eukaryotic DNA repair.
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===Solution structure of the ERCC1 central domain===
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Analysis of the XPA and ssDNA-binding surfaces on the central domain of human ERCC1 reveals evidence for subfunctionalization.,Tripsianes K, Folkers GE, Zheng C, Das D, Grinstead JS, Kaptein R, Boelens R Nucleic Acids Res. 2007;35(17):5789-98. Epub 2007 Aug 24. PMID:17720715<ref>PMID:17720715</ref>
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From MEDLINE&reg;/PubMed&reg;, a database of the U.S. National Library of Medicine.<br>
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</div>
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The line below this paragraph, {{ABSTRACT_PUBMED_17720715}}, adds the Publication Abstract to the page
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<div class="pdbe-citations 2jpd" style="background-color:#fffaf0;"></div>
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(as it appears on PubMed at http://www.pubmed.gov), where 17720715 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_17720715}}
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__TOC__
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</StructureSection>
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==Disease==
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Known disease associated with this structure: Cerebrooculofacioskeletal syndrome 4 OMIM:[[http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=126380 126380]]
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==About this Structure==
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2JPD is a [[Single protein]] structure of 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=2JPD OCA].
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==Reference==
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Analysis of the XPA and ssDNA-binding surfaces on the central domain of human ERCC1 reveals evidence for subfunctionalization., Tripsianes K, Folkers GE, Zheng C, Das D, Grinstead JS, Kaptein R, Boelens R, Nucleic Acids Res. 2007;35(17):5789-98. Epub 2007 Aug 24. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/17720715 17720715]
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[[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: Boelens, R.]]
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[[Category: Boelens R]]
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[[Category: Das, D.]]
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[[Category: Das D]]
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[[Category: Folkers, G.]]
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[[Category: Folkers G]]
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[[Category: Grinstead, J S.]]
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[[Category: Grinstead JS]]
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[[Category: Kaptein, R.]]
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[[Category: Kaptein R]]
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[[Category: Tripsianes, K.]]
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[[Category: Tripsianes K]]
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[[Category: Zheng, C.]]
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[[Category: Zheng C]]
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[[Category: Protein]]
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[[Category: Protein,dna binding protein]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Mon Jul 28 04:48:48 2008''
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Current revision

Solution structure of the ERCC1 central domain

PDB ID 2jpd

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Proteopedia Page Contributors and Editors (what is this?)

OCA

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