Helicase-deficient TFIIH causes severe disease features via persistent DNA excision without damage removal
Nucleotide excision repair (NER) removes helix-distorting DNA lesions through the ten-subunit TFIIH complex, whose XPB and XPD translocase/helicase activities unwind DNA to enable damage verification and subsequent endonucleolytic DNA incisions. While most XPD mutations cause xeroderma pigmentosum, specific helicase-de...