Excision Repair Cross Complementing Rodent Repair Deficiency Complementation 6 (ERCC6)

The ERCC6 protein is a DNA-binding protein important in transcription-coupled excision repair. The protein has ATP-stimulated ATPase activity; there are contradictory publications reporting presence or absence of helicase activity. The protein appears to interact with several transcription and excision repair proteins, and may promote complex formation at repair sites.ERCC6 gene corrected the repair defect in cells from patients with type B Cockayne syndrome, but had no effect on the UV sensitivity of cells from patients with type A Cockayne syndrome or cells from the nucleotide excision repair-defective disorder xeroderma pigmentosum. Mutation analysis of a patient with type B CS indicated that the gene is not essential for cell viability, but is specific for preferential repair of lesions from the transcribed strand of active genes.