Nucleotide excision repair (NER) is a highly conserved multi-step process. Congenital NER defects are clinically complex, at least in part because components of the NER machinery also function in the basal transcription factor, TFIIH. A new study demonstrates that reduction in the amount, rather than the inherent activity, of TFIIH is the underlying cause of one such congenital NER defect, underscoring the link between transcription and NER-associated disease.
