DNA excision repair protein ERCC-6 (ERCC6) (ERCC6)

DNA excision repair protein ERCC-6, also known as Cockayne syndrome protein B (CSB), is a critical ATPase and chromatin remodeling factor that mediates transcription-coupled nucleotide excision repair (TC-NER) (UniProt, 2024). It functions by recognizing RNA polymerase II complexes that have stalled at DNA lesions, subsequently recruiting repair factors to the site of damage to facilitate lesion removal and the resumption of transcription (MedlinePlus, 2024). Beyond its role in DNA repair, ERCC6 is involved in broader cellular processes including global transcription regulation, mitochondrial homeostasis, and the maintenance of telomere stability (Wikipedia, 2024). Mutations in the ERCC6 gene are the primary cause of Cockayne syndrome type B, a rare autosomal recessive disorder characterized by severe growth retardation, progressive neurological decline, and features of premature aging (NIH, 2024). In the context of oncology, ERCC6 is frequently overexpressed in various tumors, where it acts as a survival factor and contributes to chemoresistance against platinum-based agents and other genotoxic therapies (OncoKB, 2024). As such, ERCC6 is an attractive therapeutic target for sensitization strategies, where its inhibition can induce synthetic lethality or enhance the efficacy of DNA-damaging drugs in cancer treatment (PubMed, 2024).

Other names

CSBCKN2COFSARMD5RAD26UVSS1POF11Cockayne syndrome protein B

Inhibition of ERCC6 sensitizes cancer cells to genotoxic agents by blocking transcription-coupled nucleotide excision repair (TC-NER) and promoting apoptosis.