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:
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
Vaild Sequence:
Related Databases
Uniprot:
ELISA Kit
CLIA Kit
Polyclonal Antibody
Monoclonal Antibody
Protein
FOR
Human
Mouse
Bovine
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
R&D Technical Data
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Precision
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Recovery
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Linearity
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[
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[
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[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)
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[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)
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[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)
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[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
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[
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.
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[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.
[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.
[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.
[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.
[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.
[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.
[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]
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Nat. Genet.44:593-597(2012)
[
PubMed ]
[
Europe PMC ]
[
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[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.
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[
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[
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.
[15/1/25 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.
[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.
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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.
"Mutation update for the CSB/ERCC6 and CSA/ERCC8 genes involved in Cockayne syndrome."
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Hum. Mutat.31:113-126(2010)
[
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 : VARIANTS CSA THR-160; CYS-194; SER-202 AND GLY-266