2jnw

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{{Seed}}
 
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[[Image:2jnw.png|left|200px]]
 
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==Solution structure of a ERCC1-XPA heterodimer==
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The line below this paragraph, containing "STRUCTURE_2jnw", creates the "Structure Box" on the page.
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<StructureSection load='2jnw' size='340' side='right'caption='[[2jnw]]' 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'>[[2jnw]] is a 2 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=2JNW OCA]. For a <b>guided tour on the structure components</b> use [https://proteopedia.org/fgij/fg.htm?mol=2JNW 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=2jnw FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=2jnw OCA], [https://pdbe.org/2jnw PDBe], [https://www.rcsb.org/pdb/explore.do?structureId=2jnw RCSB], [https://www.ebi.ac.uk/pdbsum/2jnw PDBsum], [https://prosat.h-its.org/prosat/prosatexe?pdbcode=2jnw ProSAT]</span></td></tr>
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{{STRUCTURE_2jnw| PDB=2jnw | 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/jn/2jnw_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=2jnw 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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The nucleotide excision repair (NER) pathway corrects DNA damage caused by sunlight, environmental mutagens and certain antitumor agents. This multistep DNA repair reaction operates by the sequential assembly of protein factors at sites of DNA damage. The efficient recognition of DNA damage and its repair are orchestrated by specific protein-protein and protein-DNA interactions within NER complexes. We have investigated an essential protein-protein interaction of the NER pathway, the binding of the XPA protein to the ERCC1 subunit of the repair endonuclease ERCC1-XPF. The structure of ERCC1 in complex with an XPA peptide shows that only a small region of XPA interacts with ERCC1 to form a stable complex exhibiting submicromolar binding affinity. However, this XPA peptide is a potent inhibitor of NER activity in a cell-free assay, blocking the excision of a cisplatin adduct from DNA. The structure of the peptide inhibitor bound to its target site reveals a binding interface that is amenable to the development of small molecule peptidomimetics that could be used to modulate NER repair activities in vivo.
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===Solution structure of a ERCC1-XPA heterodimer===
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Structural basis for the recruitment of ERCC1-XPF to nucleotide excision repair complexes by XPA.,Tsodikov OV, Ivanov D, Orelli B, Staresincic L, Shoshani I, Oberman R, Scharer OD, Wagner G, Ellenberger T EMBO J. 2007 Nov 14;26(22):4768-76. Epub 2007 Oct 18. PMID:17948053<ref>PMID:17948053</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_17948053}}, adds the Publication Abstract to the page
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<div class="pdbe-citations 2jnw" style="background-color:#fffaf0;"></div>
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(as it appears on PubMed at http://www.pubmed.gov), where 17948053 is the PubMed ID number.
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== References ==
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<references/>
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{{ABSTRACT_PUBMED_17948053}}
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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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2JNW is a [[Protein complex]] structure of sequences 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=2JNW OCA].
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==Reference==
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Structural basis for the recruitment of ERCC1-XPF to nucleotide excision repair complexes by XPA., Tsodikov OV, Ivanov D, Orelli B, Staresincic L, Shoshani I, Oberman R, Scharer OD, Wagner G, Ellenberger T, EMBO J. 2007 Nov 14;26(22):4768-76. Epub 2007 Oct 18. PMID:[http://www.ncbi.nlm.nih.gov/pubmed/17948053 17948053]
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[[Category: Homo sapiens]]
[[Category: Homo sapiens]]
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[[Category: Protein complex]]
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[[Category: Large Structures]]
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[[Category: Ivanov, D.]]
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[[Category: Ivanov D]]
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[[Category: Orelli, B.]]
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[[Category: Orelli B]]
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[[Category: Scharer, O D.]]
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[[Category: Scharer OD]]
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[[Category: Staresincic, L.]]
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[[Category: Staresincic L]]
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[[Category: Tsodikov, O V.]]
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[[Category: Tsodikov OV]]
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[[Category: Wagner, G.]]
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[[Category: Wagner G]]
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[[Category: Dna binding protein]]
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[[Category: Ercc1]]
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[[Category: Ner]]
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[[Category: Recruitment]]
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[[Category: Xpa]]
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''Page seeded by [http://oca.weizmann.ac.il/oca OCA ] on Tue Jul 29 09:34:11 2008''
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Current revision

Solution structure of a ERCC1-XPA heterodimer

PDB ID 2jnw

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