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ERCC8 Antibody (Center)
Affinity Purified Rabbit Polyclonal Antibody (Pab)
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application 
| IHC-P, WB, E |
|---|---|
| Primary Accession | Q13216 |
| Other Accession | NP_000073.1 |
| Reactivity | Human, Mouse |
| Host | Rabbit |
| Clonality | Polyclonal |
| Isotype | Rabbit IgG |
| Calculated MW | 44055 Da |
| Antigen Region | 210-238 aa |
| Gene ID | 1161 |
|---|---|
| Other Names | DNA excision repair protein ERCC-8, Cockayne syndrome WD repeat protein CSA, ERCC8, CKN1, CSA |
| Target/Specificity | This ERCC8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 210-238 amino acids from the Central region of human ERCC8. |
| Dilution | IHC-P~~1:50~100 WB~~1:1000 E~~Use at an assay dependent concentration. |
| Format | Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification. |
| Storage | Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles. |
| Precautions | ERCC8 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures. |
| Name | ERCC8 {ECO:0000303|PubMed:19894250, ECO:0000312|HGNC:HGNC:3439} |
|---|---|
| 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 (TC-NER), a process during which RNA polymerase II-blocking lesions are rapidly removed from the transcribed strand of active genes (PubMed:12732143, PubMed:16751180, PubMed:16964240, PubMed:32142649, PubMed:34526721, PubMed:38316879, PubMed:38600235, PubMed:38600236). Following recruitment to lesion-stalled RNA polymerase II (Pol II), the CSA complex mediates ubiquitination of Pol II subunit POLR2A/RPB1 at 'Lys- 1268', a critical TC-NER checkpoint, governing RNA Pol II stability and initiating DNA damage excision by TFIIH recruitment (PubMed:12732143, PubMed:16751180, PubMed:16964240, PubMed:32142649, PubMed:32355176, PubMed:34526721, PubMed:38316879, PubMed:38600235, PubMed:38600236). The CSA complex also promotes the ubiquitination and subsequent proteasomal degradation of ERCC6/CSB in a UV-dependent manner; ERCC6 degradation is essential for the recovery of RNA synthesis after transcription-coupled repair (PubMed:16751180). Also plays a role in DNA double-strand breaks (DSSBs) repair by non-homologous end joining (NHEJ) (PubMed:29545921). |
| Cellular Location | Nucleus. Chromosome Nucleus matrix. Note=Recruited to lesion- stalled RNA polymerase II (Pol II) sites by ERCC6/CSB (PubMed:32355176). UV-induced translocation to the nuclear matrix is dependent on ERCC6/CSB (PubMed:26620705). |
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Provided below are standard protocols that you may find useful for product applications.
Background
This gene 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. Mutations in this gene have been identified in patients with hereditary disease Cockayne syndrome (CS). CS cells are abnormally sensitive to ultraviolet radiation and are defective in the repair of transcriptionally active genes.
References
Briggs, F.B., et al. Am. J. Epidemiol. 172(2):217-224(2010)
Guillem, V.M., et al. Am. J. Hematol. 85(7):482-486(2010)
Monsees, G.M., et al. Breast Cancer Res. Treat. (2010) In press :
Kamenisch, Y., et al. J. Exp. Med. 207(2):379-390(2010)
Laugel, V., et al. Hum. Mutat. 31(2):113-126(2010)
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