DNA excision repair protein ERCC-8 (ERCC8) is a ~44 kDa protein. 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. 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. 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. Also plays a role in DNA double-strand breaks (DSSBs) repair by non-homologous end joining (NHEJ). 2. Groisman, R. et al. (2006) Genes & development 20, 1429-34. PMID: 167511806. Kokic, G. et al. (2024) Nature structural & molecular biology 31, 536-547. PMID: 383168797. Carnie, CJ. et al. (2024) Nature cell biology 26, 797-810. PMID: 386002358. van Sluis, M. et al. (2024) Nature cell biology 26, 770-783. PMID: 386002369. van der Weegen, Y. et al. (2020) Nature communications 11, 2104. PMID: 32355176 | 
