Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8)
[Edit]CKN1; CSA; Cockayne Syndrome 1; Cockayne syndrome WD repeat protein CSA; DNA excision repair protein ERCC-8
![Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8)](/upload/item/img/Excision-Repair-Cross-Complementing-Rodent-Repair-Deficiency-Complementation-8-(ERCC8)-94729.jpg)
ERCC8 encodes a WD repeat protein, which interacts with Cockayne syndrome type B (CSB) protein and with p44 protein, a subunit of the RNA polymerase II transcription factor IIH.Multiple alternatively spliced transcript variants encoding different isoforms have been found for this gene.In vitro translated CSA protein specifically interacted with the CSB (ERCC6) protein and p44 protein (GTF2H2), a subunit of the RNA polymerase II basal transcription factor TFIIH. These observations, coupled with the observation that the amino acid sequence of the predicted CSB polypeptide is homologous with a component of the yeast Swi/Snf transcriptional activation complex, suggested that Cockayne syndrome cells are defective in RNA polymerase II transcription.
Organism species: Homo sapiens (Human)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | RPE729Hu01 | Recombinant Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | n/a | Monoclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | ELISA Kit Customized Service Offer |
Organism species: Mus musculus (Mouse)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | RPE729Mu01 | Recombinant Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Positive Control; Immunogen; SDS-PAGE; WB. |
Antibodies | n/a | Monoclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | ELISA Kit Customized Service Offer |
Organism species: Rattus norvegicus (Rat)
CATALOG NO. | PRODUCT NAME | APPLICATIONS | |
Proteins | n/a | Recombinant Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Recombinant Protein Customized Service Offer |
Antibodies | n/a | Monoclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Monoclonal Antibody Customized Service Offer |
n/a | Polyclonal Antibody to Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | Polyclonal Antibody Customized Service Offer | |
Assay Kits | n/a | CLIA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | CLIA Kit Customized Service Offer |
n/a | ELISA Kit for Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 8 (ERCC8) | ELISA Kit Customized Service Offer |
- "The Cockayne syndrome group A gene encodes a WD repeat protein that interacts with CSB protein and a subunit of RNA polymerase II TFIIH."Cell 82:555-564(1995) [PubMed] [Europe PMC] [Abstract]
- "Complete sequencing and characterization of 21,243 full-length human cDNAs." Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
- "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
- "The ubiquitin ligase activity in the DDB2 and CSA complexes is differentially regulated by the COP9 signalosome in response to DNA damage."Cell 113:357-367(2003) [PubMed] [Europe PMC] [Abstract]
- "CSA-dependent degradation of CSB by the ubiquitin-proteasome pathway establishes a link between complementation factors of the Cockayne syndrome."Genes Dev. 20:1429-1434(2006) [PubMed] [Europe PMC] [Abstract]
- "Cockayne syndrome A and B proteins differentially regulate recruitment of chromatin remodeling and repair factors to stalled RNA polymerase II in vivo."Mol. Cell 23:471-482(2006) [PubMed] [Europe PMC] [Abstract]
- "Molecular architecture and assembly of the DDB1-CUL4A ubiquitin ligase machinery."Nature 443:590-593(2006) [PubMed] [Europe PMC] [Abstract]
- "System-wide temporal characterization of the proteome and phosphoproteome of human embryonic stem cell differentiation."Sci. Signal. 4:RS3-RS3(2011) [PubMed] [Europe PMC] [Abstract]
- "Mutations in UVSSA cause UV-sensitive syndrome and destabilize ERCC6 in transcription-coupled DNA repair."Nat. Genet. 44:593-597(2012) [PubMed] [Europe PMC] [Abstract]
- "The molecular basis of CRL4DDB2/CSA ubiquitin ligase architecture, targeting, and activation."Cell 147:1024-1039(2011) [PubMed] [Europe PMC] [Abstract]
- "CKN1 (MIM 216400): mutations in Cockayne syndrome type A and a new common polymorphism."J. Hum. Genet. 49:61-63(2004) [PubMed] [Europe PMC] [Abstract]
- "Characterisation of novel mutations in Cockayne syndrome type A and xeroderma pigmentosum group C subjects."J. Hum. Genet. 50:151-154(2005) [PubMed] [Europe PMC] [Abstract]
- "A UV-sensitive syndrome patient with a specific CSA mutation reveals separable roles for CSA in response to UV and oxidative DNA damage."Proc. Natl. Acad. Sci. U.S.A. 106:6209-6214(2009) [PubMed] [Europe PMC] [Abstract]
- "Mutation update for the CSB/ERCC6 and CSA/ERCC8 genes involved in Cockayne syndrome." Hum. Mutat. 31:113-126(2010) [PubMed] [Europe PMC] [Abstract]