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Index > Protein center > ERCC8(Gene name) > Human
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  • ERCC8 (Gene name),
  • DNA excision repair protein ERCC-8 (Protein name ),  ERCC8_HUMAN from NCBI database.
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  • General Annotation
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  • Antigen Annotation
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  • 3D
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  • Predicted Eptitope
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  • Vaild Sequence
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  • Gene name:
    ERCC8(CKN1;CSA);
    Protein name:
    DNA excision repair protein ERCC-8;
    Alternative:
    Cockayne syndrome WD repeat protein CSA;
    Organism:
    Human (Homo sapiens). 
    General Annotation
    Sub Unit:
    Part of the CSA complex (DCX(ERCC8) complex), a DCX E3 ubiquitin-protein ligase complex containing ERCC8, RBX1, DDB1 and CUL4A; the CSA complex interacts with RNA polymerase II; upon UV irradiation it interacts with the COP9 signalosome and preferentially with the hyperphosphorylated form of RNA polymerase II. Interacts with ERCC6. Interacts with a subunit of RNA polymerase II TFIIH. Interacts with DDB1.
    Function:
    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 recruitement 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.
    Subcellular Location:
    Nucleus
    Protein Attributes:
    Sequence length:
    396
    Sequence:
    50:
    MLGFLSARQT | GLEDPLRLRR | AESTRRVLGL | ELNKDRDVER | IHGGGINTLD | 
    100:
    IEPVEGRYML | SGGSDGVIVL | YDLENSSRQS | YYTCKAVCSI | GRDHPDVHRY | 
    150:
    SVETVQWYPH | DTGMFTSSSF | DKTLKVWDTN | TLQTADVFNF | EETVYSHHMS | 
    200:
    PVSTKHCLVA | VGTRGPKVQL | CDLKSGSCSH | ILQGHRQEIL | AVSWSPRYDY | 
    250:
    ILATASADSR | VKLWDVRRAS | GCLITLDQHN | GKKSQAVESA | NTAHNGKVNG | 
    300:
    LCFTSDGLHL | LTVGTDNRMR | LWNSSNGENT | LVNYGKVCNN | SKKGLKFTVS | 
    350:
    CGCSSEFVFV | PYGSTIAVYT | VYSGEQITML | KGHYKTVDCC | VFQSNFQELY | 
    396:
    SGSRDCNILA | WVPSLYEPVP | DDDETTTKSQ | LNPAFEDAWS | SSDEEG
    3D Structure:
    N/A
    Predicted Eptitope:
    Please Sign in.
    EIAab Sequence  Vaild Sequence:
    Please Sign in.
    Related Databases
    String:
    UniGene:
    KEGG:
    Pfam:
    SMR:
    MIM:
    Uniprot:
     
    FOR
    ELISA Kit for Human DNA excision repair protein ERCC-8
    ELISA Kit for Human DNA excision repair protein ERCC-8
    ELISA Kit for Human DNA excision repair protein ERCC-8
    CLIA Kit for Human DNA excision repair protein ERCC-8
    CLIA Kit for Human DNA excision repair protein ERCC-8
    CLIA Kit for Human DNA excision repair protein ERCC-8
    Polyclonal Antibody for Human DNA excision repair protein ERCC-8
    Polyclonal Antibody for Human DNA excision repair protein ERCC-8
    Polyclonal Antibody for Human DNA excision repair protein ERCC-8
    Monoclonal Antibody for Human DNA excision repair protein ERCC-8
    Monoclonal Antibody for Human DNA excision repair protein ERCC-8
    Monoclonal Antibody for Human DNA excision repair protein ERCC-8
    Protein for Human DNA excision repair protein ERCC-8
    Protein for Human DNA excision repair protein ERCC-8
    Protein for Human DNA excision repair protein ERCC-8

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    Linearity
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    References
    1. 1.
      "The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH."
      Henning K.A. , Li L. , Iyer N. , McDaniel L.D. , Reagan M.S. , Legerski R. , Schultz R.A. , Stefanini M. , Lehmann A.R. , Mayne L.V. , Friedberg E.C.
      Cell82:555-564(1995) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1)
    2. 2.
      "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
      Halleck A. , Ebert L. , Mkoundinya M. , Schick M. , Eisenstein S. , Neubert P. , Kstrang K. , Schatten R. , Shen B. , Henze S. , Mar W. , Korn B. , Zuo D. , Hu Y. , LaBaer J.
      Submitted (2004-06) to the EMBL/GenBank/DDBJ databases
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1)
    3. 3.
      "Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
      Kalnine N. , Chen X. , Rolfs A. , Halleck A. , Hines L. , Eisenstein S. , Koundinya M. , Raphael J. , Moreira D. , Kelley T. , LaBaer J. , Lin Y. , Phelan M. , Farmer A.
      Submitted (2004-10) to the EMBL/GenBank/DDBJ databases
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1)
    4. 4.
      NIEHS SNPs program
      Submitted (2003-01) to the EMBL/GenBank/DDBJ databases
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA];VARIANT CYS-200
    5. 5.
      "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T. , Suzuki Y. , Nishikawa T. , Otsuki T. , Sugiyama T. , Irie R. , Wakamatsu A. , Hayashi K. , Sato H. , Nagai K. , Kimura K. , Makita H. , Sekine M. , Obayashi M. , Nishi T. , Shibahara T. , Tanaka T. , Ishii S. , more...
      Nat. Genet.36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1)
      tissue: Uterus.
    6. 6.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA]
    7. 7.
      "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res.14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 2)
      tissue: Urinary bladder.
    8. 8.
      "The ubiquitin ligase activity in the DDB2 and CSA complexes is differentially regulated by the COP9 signalosome in response to DNA damage."
      Groisman R. , Polanowska J. , Kuraoka I. , Sawada J. , Saijo M. , Drapkin R. , Kisselev A.F. , Tanaka K. , Nakatani Y.
      Cell113:357-367(2003) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH DDB1;IDENTIFICATION IN THE CSA COMPLEX WITH RBX1; DDB1 AND CUL4A;INTERACTION OF THE CSA COMPLEX WITH RNA POLYMERASE II AND THE COP9 SIGNALOSOME
    9. 9.
      "CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome."
      Groisman R. , Kuraoka I. , Chevallier O. , Gaye N. , Magnaldo T. , Tanaka K. , Kisselev A.F. , Harel-Bellan A. , Nakatani Y.
      Genes Dev.20:1429-1434(2006) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH ERCC6;FUNCTION AS SUBSTRATE-RECOGNITION COMPONENT OF THE CSA COMPLEX
    10. 10.
      "Cockayne syndrome A and B proteins differentially regulate recruitment of chromatin remodeling and repair factors to stalled RNA polymerase II in vivo."
      Fousteri M. , Vermeulen W. , van Zeeland A.A. , Mullenders L.H.
      Mol. Cell23:471-482(2006) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: FUNCTION
    11. 11.
      "Molecular architecture and assembly of the DDB1-CUL4A ubiquitin ligase machinery."
      Angers S. , Li T. , Yi X. , MacCoss M.J. , Moon R.T. , Zheng N.
      Nature443:590-593(2006) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: FUNCTION
    12. 12.
      "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."
      Rigbolt K.T. , Prokhorova T.A. , Akimov V. , Henningsen J. , Johansen P.T. , Kratchmarova I. , Kassem M. , Mann M. , Olsen J.V. , Blagoev B.
      Sci. Signal.4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: PHOSPHORYLATION [LARGE SCALE ANALYSIS] AT SER-390; SER-391 AND SER-392;IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS]
    13. 13.
      "Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair."
      Zhang X. , Horibata K. , Saijo M. , Ishigami C. , Ukai A. , Kanno S.I. , Tahara H. , Neilan E.G. , Honma M. , Nohmi T. , Yasui A. , Tanaka K.
      Nat. Genet.44:593-597(2012) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: INTERACTION WITH UVSSA
    14. 14.
      "The molecular basis of CRL4DDB2/CSA ubiquitin ligase architecture, targeting, and activation."
      Fischer E.S. , Scrima A. , Bohm K. , Matsumoto S. , Lingaraju G.M. , Faty M. , Yasuda T. , Cavadini S. , Wakasugi M. , Hanaoka F. , Iwai S. , Gut H. , Sugasawa K. , Thoma N.H.
      Cell147:1024-1039(2011) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: X-RAY CRYSTALLOGRAPHY (3.31 ANGSTROMS) IN COMPLEX WITH DDB1;SUBUNIT
    15. 15.
      "CKN1 (MIM 216400): mutations in Cockayne syndrome type A and a new common polymorphism."
      Cao H. , Williams C. , Carter M. , Hegele R.A.
      J. Hum. Genet.49:61-63(2004) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT CSA PRO-205
    16. 16.
      "Characterisation of novel mutations in Cockayne syndrome type A and xeroderma pigmentosum group C subjects."
      Ridley A.J. , Colley J. , Wynford-Thomas D. , Jones C.J.
      J. Hum. Genet.50:151-154(2005) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT CSA VAL-160
    17. 17.
      "A UV-sensitive syndrome patient with a specific CSA mutation reveals separable roles for CSA in response to UV and oxidative DNA damage."
      Nardo T. , Oneda R. , Spivak G. , Vaz B. , Mortier L. , Thomas P. , Orioli D. , Laugel V. , Stary A. , Hanawalt P.C. , Sarasin A. , Stefanini M.
      Proc. Natl. Acad. Sci. U.S.A.106:6209-6214(2009) [PubMed] [Europe PMC] [Abstract]
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANT UVSS2 CYS-361
    18. 18.
      [15/1/25 17:38] Upload to ab completed in less than a minute: 1 file transferred (13.4 Kb/s) Cited for: VARIANTS CSA THR-160; CYS-194; SER-202 AND GLY-266
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