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'''Unreleased structure'''


The entry 6fcv is ON HOLD
==Structure of the human DDB1-CSA complex==
 
<StructureSection load='6fcv' size='340' side='right' caption='[[6fcv]], [[Resolution|resolution]] 2.92&Aring;' scene=''>
Authors: Meulenbroek, E.M., Pannu, N.S.
== Structural highlights ==
 
<table><tr><td colspan='2'>[[6fcv]] is a 2 chain structure. Full crystallographic information is available from [http://oca.weizmann.ac.il/oca-bin/ocashort?id=6FCV OCA]. For a <b>guided tour on the structure components</b> use [http://oca.weizmann.ac.il/oca-docs/fgij/fg.htm?mol=6FCV FirstGlance]. <br>
Description: Structure of the human DDB1-CSA complex
</td></tr><tr id='related'><td class="sblockLbl"><b>[[Related_structure|Related:]]</b></td><td class="sblockDat">[[4a11|4a11]]</td></tr>
[[Category: Unreleased Structures]]
<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=6fcv FirstGlance], [http://oca.weizmann.ac.il/oca-bin/ocaids?id=6fcv OCA], [http://pdbe.org/6fcv PDBe], [http://www.rcsb.org/pdb/explore.do?structureId=6fcv RCSB], [http://www.ebi.ac.uk/pdbsum/6fcv PDBsum], [http://prosat.h-its.org/prosat/prosatexe?pdbcode=6fcv ProSAT]</span></td></tr>
[[Category: Meulenbroek, E.M]]
</table>
[[Category: Pannu, N.S]]
== Disease ==
[[http://www.uniprot.org/uniprot/ERCC8_HUMAN ERCC8_HUMAN]] Cockayne syndrome type 1;Cockayne syndrome type 3;Cockayne syndrome type 2;UV-sensitive 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.  
== Function ==
[[http://www.uniprot.org/uniprot/DDB1_HUMAN DDB1_HUMAN]] Required for DNA repair. Binds to DDB2 to form the UV-damaged DNA-binding protein complex (the UV-DDB complex). The UV-DDB complex may recognize UV-induced DNA damage and recruit proteins of the nucleotide excision repair pathway (the NER pathway) to initiate DNA repair. The UV-DDB complex preferentially binds to cyclobutane pyrimidine dimers (CPD), 6-4 photoproducts (6-4 PP), apurinic sites and short mismatches. Also appears to function as a component of numerous distinct DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complexes which mediate the ubiquitination and subsequent proteasomal degradation of target proteins. The functional specificity of the DCX E3 ubiquitin-protein ligase complex is determined by the variable substrate recognition component recruited by DDB1. DCX(DDB2) (also known as DDB1-CUL4-ROC1, CUL4-DDB-ROC1 and CUL4-DDB-RBX1) may ubiquitinate histone H2A, histone H3 and histone H4 at sites of UV-induced DNA damage. The ubiquitination of histones may facilitate their removal from the nucleosome and promote subsequent DNA repair. DCX(DDB2) also ubiquitinates XPC, which may enhance DNA-binding by XPC and promote NER. DCX(DTL) plays a role in PCNA-dependent polyubiquitination of CDT1 and MDM2-dependent ubiquitination of TP53 in response to radiation-induced DNA damage and during DNA replication. DCX(ERCC8) (the CSA complex) plays a role in transcription-coupled repair (TCR). May also play a role in ubiquitination of CDKN1B/p27kip when associated with CUL4 and SKP2.<ref>PMID:12732143</ref> <ref>PMID:15448697</ref> <ref>PMID:14739464</ref> <ref>PMID:15882621</ref> <ref>PMID:16260596</ref> <ref>PMID:16482215</ref> <ref>PMID:17079684</ref> <ref>PMID:16407242</ref> <ref>PMID:16407252</ref> <ref>PMID:16678110</ref> <ref>PMID:16940174</ref> <ref>PMID:17041588</ref> <ref>PMID:16473935</ref> <ref>PMID:18593899</ref> <ref>PMID:18381890</ref> <ref>PMID:18332868</ref>  [[http://www.uniprot.org/uniprot/ERCC8_HUMAN ERCC8_HUMAN]] Substrate-recognition component of the CSA complex, a DCX (DDB1-CUL4-X-box) E3 ubiquitin-protein ligase complex, involved in transcription-coupled nucleotide excision repair. The CSA complex (DCX(ERCC8) complex) promotes the ubiquitination and subsequent proteasomal degradation of ERCC6 in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair. It is required for the recruitment of XAB2, HMGN1 and TCEA1/TFIIS to a transcription-coupled repair complex which removes RNA polymerase II-blocking lesions from the transcribed strand of active genes.<ref>PMID:16751180</ref> <ref>PMID:16916636</ref> <ref>PMID:16964240</ref> 
== References ==
<references/>
__TOC__
</StructureSection>
[[Category: Meulenbroek, E M]]
[[Category: Pannu, N S]]
[[Category: Dna damage response protein]]
[[Category: Protein binding]]
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