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

DNA excision repair protein ERCC-6 (CSB, encoded by the ERCC6 gene) is an ATP-dependent chromatin remodeling helicase essential for **transcription-coupled nucleotide excision repair**—a DNA repair pathway that rapidly removes DNA lesions from the transcribed strand of active genes[1][2][3]. The CSB protein also assists in other DNA repair mechanisms such as base excision repair, and it helps restart transcription after DNA damage via chromatin remodeling and by removing stalled RNA polymerase from sites of DNA lesions[1][2][5]. Mutations in ERCC6 cause autosomal recessive disorders like Cockayne syndrome type B, UV-sensitive syndrome, and have been associated with age-related macular degeneration and other developmental or repair disorders[2][4]. CSB exhibits ATPase activity and interacts with several proteins (such as RNA polymerase II, NAP1, and DNA glycosylases) to remodel chromatin structure and coordinate DNA repair enzyme access to damaged DNA[5]. There are currently no drugs that directly target ERCC6/CSB, making it primarily a **tool for genetic diagnosis** and basic research rather than a therapeutic target.

Other names

Cockayne syndrome protein BCSBexcision repair cross-complementation group 6RAD26ARMD5CKN2COFSCOFS1POF11UVSS1Chimeric ERCC6-PGBD3 proteinChimeric CSB-PGBD3 proteinCSB-PGBD3ERCC6-PGBD3 fusion proteinERCC6_HUMAN

Not applicable; no approved or investigational therapeutics directly target ERCC6/CSB