When more is better

Nucleotide excision repair (NER) is a highly conserved multi-step process. Congenital NER defects are clinically complex, at least in part because com ponents of the NER machinery also function in the basal transcription facto r, TFIIH. A new study demonstrates that reduction in the amount, rather tha n the inherent activity, of TFIIH is the underlying cause of one such conge nital NER defect, underscoring the link between transcription and NER-assoc iated disease.