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Rabbit Anti-ERCC5 Recombinant Antibody (EG1132) (CBMAB-EN1349-LY)


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Summary

Host Animal
Rabbit
Specificity
Human
Clone
EG1132
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 ELISA: 1:5000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human ERCC5.
Specificity
Human
Antibody Isotype
IgG
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!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
ERCC Excision Repair 5, Endonuclease
Introduction
This gene encodes a single-strand specific DNA endonuclease that makes the 3' incision in DNA excision repair following UV-induced damage. The protein may also function in other cellular processes, including RNA polymerase II transcription, and transcription-coupled DNA repair. Mutations in this gene cause xeroderma pigmentosum complementation group G (XP-G), which is also referred to as xeroderma pigmentosum VII (XP7), a skin disorder characterized by hypersensitivity to UV light and increased susceptibility for skin cancer development following UV exposure. Some patients also develop Cockayne syndrome, which is characterized by severe growth defects, cognitive disability, and cachexia. Read-through transcription exists between this gene and the neighboring upstream BIVM (basic, immunoglobulin-like variable motif containing) gene. [provided by RefSeq, Feb 2011]
Entrez Gene ID
2073
UniProt ID
P28715
Alternative Names
ERCC Excision Repair 5, Endonuclease; Excision Repair Cross-Complementing Rodent Repair Deficiency, Complementation Group 5; Xeroderma Pigmentosum, Complementation Group G; Excision Repair Cross-Complementation Group 5; DNA Excision Repair Protein ERCC-5; ERCM2; XPGC; XPG; Xeroderma Pigmentosum Group G-Complementing Protein;
Research Area
Single-stranded structure-specific DNA endonuclease involved in DNA excision repair (PubMed:8206890, PubMed:8090225, PubMed:8078765, PubMed:7651464, PubMed:32821917, PubMed:32522879).

Makes the 3'incision in DNA nucleotide excision repair (NER) (PubMed:8090225, PubMed:8078765, PubMed:32821917, PubMed:32522879).

Binds and bends DNA repair bubble substrate and breaks base stacking at the single-strand/double-strand DNA junction of the DNA bubble (PubMed:32522879).

Plays a role in base excision repair (BER) by promoting the binding of DNA glycosylase NTHL1 to its substrate and increasing NTHL1 catalytic activity that removes oxidized pyrimidines from DNA (PubMed:9927729).

Involved in transcription-coupled nucleotide excision repair (TCR) which allows RNA polymerase II-blocking lesions to be rapidly removed from the transcribed strand of active genes (PubMed:16246722).

Functions during the initial step of TCR in cooperation with ERCC6/CSB to recognized stalled RNA polymerase II (PubMed:16246722).

Also, stimulates ERCC6/CSB binding to the DNA repair bubble and ERCC6/CSB ATPase activity (PubMed:16246722).

Required for DNA replication fork maintenance and preservation of genomic stability (PubMed:26833090, PubMed:32522879).

Involved in homologous recombination repair (HRR) induced by DNA replication stress by recruiting RAD51, BRCA2, and PALB2 to the damaged DNA site (PubMed:26833090).

During HRR, binds to the replication fork with high specificity and stabilizes it (PubMed:32522879).

Also, acts upstream of HRR, to promote the release of BRCA1 from DNA (PubMed:26833090).
Biological Process
Base-excision repair, AP site formation Source: UniProtKB
Double-strand break repair via homologous recombination Source: UniProtKB
Negative regulation of apoptotic process Source: UniProtKB
Nucleotide-excision repair Source: UniProtKB
Nucleotide-excision repair, DNA incision, 3'-to lesion Source: UniProtKB
Nucleotide-excision repair, DNA incision, 5'-to lesion Source: Reactome
Regulation of catalytic activity Source: UniProtKB
Response to UV Source: UniProtKB
Response to UV-C Source: UniProtKB
Transcription-coupled nucleotide-excision repair Source: UniProtKB
UV protection Source: MGI
Cellular Location
Nucleus; Chromosome. Colocalizes with RAD51 to nuclear foci in S phase (PubMed:26833090). Localizes to DNA double-strand breaks (DBS) during replication stress (PubMed:26833090). Colocalizes with BRCA2 to nuclear foci following DNA replication stress (PubMed:26833090).
Involvement in disease
Xeroderma pigmentosum complementation group G (XP-G):
An autosomal recessive pigmentary skin disorder characterized by solar hypersensitivity of the skin, high predisposition for developing cancers on areas exposed to sunlight and, in some cases, neurological abnormalities. The skin develops marked freckling and other pigmentation abnormalities. Some XP-G patients present features of Cockayne syndrome, cachectic dwarfism, pigmentary retinopathy, ataxia, decreased nerve conduction velocities. The phenotype combining xeroderma pigmentosum and Cockayne syndrome traits is referred to as XP-CS complex.
Cerebro-oculo-facio-skeletal syndrome 3 (COFS3):
A disorder of prenatal onset characterized by microcephaly, congenital cataracts, facial dysmorphism, neurogenic arthrogryposis, growth failure and severe psychomotor retardation. COFS is considered to be part of the nucleotide-excision repair disorders spectrum that include also xeroderma pigmentosum, trichothiodystrophy and Cockayne syndrome.
More Infomation

Abdalkhalek, E. S., Wakeel, L. M. E., Nagy, A. A., & Sabri, N. A. (2022). Variants of ERCC5 and the outcome of platinum-based regimens in non-small cell lung cancer: a prospective cohort study. Medical Oncology, 39(10), 1-9.

Das, A. P., Saini, S., Tyagi, S., Chaudhary, N., & Agarwal, S. M. (2022). Elucidation of Increased Cervical Cancer Risk Due to Polymorphisms in XRCC1 (R399Q and R194W), ERCC5 (D1104H), and NQO1 (P187S). Reproductive Sciences, 1-15.

Bai, Z., Luo, Y., & Tian, L. (2022). ERCC5, HES6 and RORA are potential diagnostic markers of coronary artery disease. FEBS Open Bio.

Pongsavee, M. (2022). Effects of ERCC5 rs751402 Polymorphism on Oxidative Stress and the Impact of Curcumin on Catalase Activity in Breast Carcinogenesis. Asian Pacific Journal of Cancer Prevention, 23(6), 2065-2070.

Moura, D. S., Sanchez-Bustos, P., Fernandez-Serra, A., Lopez-Alvarez, M., Mondaza-Hernandez, J. L., Blanco-Alcaina, E., ... & Martin-Broto, J. (2020). CUL4A, ERCC5, and ERCC1 as predictive factors for trabectedin efficacy in advanced soft tissue sarcomas (STS): a Spanish Group for Sarcoma Research (GEIS) Study. Cancers, 12(5), 1128.

Yang, G., Yang, Y., Ma, X., Huang, L., Li, W., Song, X., ... & Lu, J. (2020). Association of ERCC5 genetic polymorphisms with cirrhosis and liver cancer. Technology in Cancer Research & Treatment, 19, 1533033820943244.

Malik, S. S., Mubarik, S., Baig, M., Masood, N., & Chaudhry, N. (2019). Genetic polymorphism in ERCC5 and breast cancer risk. Molecular Biology Research Communications, 8(1), 27.

Chikhaoui, A., Elouej, S., Nabouli, I., Jones, M., Lagarde, A., Ben Rekaya, M., ... & Yacoub-Youssef, H. (2019). Identification of a ERCC5 c. 2333T> C (L778P) variant in two Tunisian siblings with mild xeroderma pigmentosum phenotype. Frontiers in genetics, 10, 111.

Pongsavee, M., & Wisuwan, K. (2018). ERCC5 rs751402 polymorphism is the risk factor for sporadic breast cancer in Thailand. International Journal of Molecular Epidemiology and Genetics, 9(4), 27.

Liu, Y., Hu, Y., Zhang, M., Jiang, R., & Liang, C. (2018). Polymorphisms in ERCC2 and ERCC5 and risk of prostate cancer: a meta-analysis and systematic review. Journal of Cancer, 9(16), 2786.

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

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