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Mouse Anti-ERCC8 Recombinant Antibody (CBCNC-593) (CBMAB-C3424-CN)

This product is a Mouse antibody that recognizes ERCC8. The antibody CBCNC-593 can be used for immunoassay techniques such as: WB.
See all ERCC8 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBCNC-593
Antibody Isotype
IgG1
Application
WB

Basic Information

Specificity
Human
Antibody Isotype
IgG1
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Buffer
PBS, pH 7.4, 1% BSA
Preservative
0.05% Sodium azide

Target

Full Name
ERCC Excision Repair 8, CSA Ubiquitin Ligase Complex Subunit
Introduction
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. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Mar 2014]
Entrez Gene ID
UniProt ID
Alternative Names
ERCC Excision Repair 8, CSA Ubiquitin Ligase Complex Subunit; Excision Repair Cross-Complementing Rodent Repair Deficiency, Complementation Group 8; Excision Repair Cross-Complementation Group 8; Cockayne Syndrome WD Repeat Protein CSA; CKN1; CSA;
Research Area
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. Plays a role in DNA single-strand and double-strand breaks (DSSBs) repair; involved in repair of DSSBs by non-homologous end joining (NHEJ) (PubMed:29545921).
Biological Process
Cellular response to DNA damage stimulus Source: UniProtKB
DNA duplex unwinding Source: GOC
Double-strand break repair via classical nonhomologous end joining Source: UniProtKB
Nucleotide-excision repair Source: UniProtKB
Positive regulation of DNA repair Source: UniProtKB
Proteasome-mediated ubiquitin-dependent protein catabolic process Source: UniProtKB
Protein autoubiquitination Source: UniProtKB
Protein polyubiquitination Source: UniProtKB
Response to oxidative stress Source: UniProtKB
Response to UV Source: UniProtKB
Single strand break repair Source: UniProtKB
Transcription-coupled nucleotide-excision repair Source: MGI
Cellular Location
Nucleus; Nucleus matrix. UV-induced translocation to the nuclear matrix is dependent on ERCC6.
Involvement in disease
Cockayne syndrome A (CSA):
A rare disorder characterized by cutaneous sensitivity to sunlight, abnormal and slow growth, cachectic dwarfism, progeroid appearance, progressive pigmentary retinopathy and sensorineural deafness. There is delayed neural development and severe progressive neurologic degeneration resulting in mental retardation. Two clinical forms are recognized: in the classical form or Cockayne syndrome type 1, the symptoms are progressive and typically become apparent within the first few years or life; the less common Cockayne syndrome type 2 is characterized by more severe symptoms that manifest prenatally. Cockayne syndrome shows some overlap with certain forms of xeroderma pigmentosum. Unlike xeroderma pigmentosum, patients with Cockayne syndrome do not manifest increased freckling and other pigmentation abnormalities in the skin and have no significant increase in skin cancer.
UV-sensitive syndrome 2 (UVSS2):
An autosomal recessive disorder characterized by cutaneous photosensitivity and mild freckling in the absence of neurological abnormalities or skin tumors.

Mamoor, S. (2022). A single nucleotide variant on chromosome 5 residing within ERCC8 distinguishes patients with basal-like human breast cancer.

Duong, N. T., Dinh, T. H., Möhl, B. S., Hintze, S., Ha, D. T. T., Ngoc, N. D., ... & Meinke, P. (2022). Cockayne syndrome without UV-sensitivity in Vietnamese siblings with novel ERCC8 variants. Aging (Albany NY), 14(13), 5299.

Gauhar, Z., Tejwani, L., Abdullah, U., Saeed, S., Shafique, S., Badshah, M., ... & Raja, G. K. (2022). A Novel Missense Mutation in ERCC8 Co-Segregates with Cerebellar Ataxia in a Consanguineous Pakistani Family. Cells, 11(19), 3090.

Sui, X., Tang, X., Wu, X., & Liu, Y. (2022). Identification of ERCC8 as a novel cisplatin-resistant gene in esophageal cancer based on genome-scale CRISPR/Cas9 screening. Biochemical and Biophysical Research Communications, 593, 84-92.

Yousefipour, F., & Mahjoobi, F. (2021). Identification of two novel homozygous mutations in ERCC8 gene in two unrelated consanguineous families with Cockayne syndrome from Iran. Clinica Chimica Acta, 523, 65-71.

Chen, J., Li, L., Sun, L., Yuan, Y., & Jing, J. (2021). Associations of individual and joint expressions of ERCC6 and ERCC8 with clinicopathological parameters and prognosis of gastric cancer. PeerJ, 9, e11791.

Moslehi, R., Tsao, H. S., Zeinomar, N., Stagnar, C., Fitzpatrick, S., & Dzutsev, A. (2020). Integrative genomic analysis implicates ERCC6 and its interaction with ERCC8 in susceptibility to breast cancer. Scientific reports, 10(1), 1-13.

Zhang, D., Dai, L., Zhou, Z., Hu, J., Bai, Y., & Guo, H. (2019). Homozygosity mapping and whole exome sequencing reveal a novel ERCC8 mutation in a Chinese consanguineous family with unique cerebellar ataxia. Clinica Chimica Acta, 494, 64-70.

Lu, X., Chen, F., Liu, X., Yuan, D., Zi, Y., He, X., & Zhu, D. (2018). Detection and clinical significance of DNA repair gene ERCC8 tag SNPs in gastric cancer. The Turkish Journal of Gastroenterology, 29(4), 392.

Chebly, A., Corbani, S., Abou Ghoch, J., Mehawej, C., Megarbane, A., & Chouery, E. (2018). First molecular study in Lebanese patients with Cockayne syndrome and report of a novel mutation in ERCC8 gene. BMC medical genetics, 19(1), 1-7.

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For research use only. Not intended for any clinical use.

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