ERCC6, also known as Cockayne syndrome B (CSB), is a member of the SWI2/SNF2 family of chromatin remodeling complexes [368], involved in mitochondrial DNA (mtDNA) damage repair, base excision repair (BER), interstrand crosslink (ICL) repair, and double-strand break (DSB) repair. This evidence concerns the gene ERCC6 and Cockayne syndrome.