This entry represents the helicase Ercc3 (also known as RepB/XPB/Rad25/Ssl2/haywire). Ercc3 is a component of the core-TFIIH basal transcription factor involved in nucleotide excision repair (NER) of DNA and in RNA transcription by RNA polymerase II [1]. The TFIIH multiprotein complex consists of a 7-subunit core (XPB, p62, p52, p44, p34, and TTDA) that is associated with a 3-subunit CDK-activating kinase module (MAT1, cyclin H and Cdk7) [2]. Errc3 acts by opening DNA either around the RNA transcription start site or the DNA damage [3].

Defects in ERCC3 are the cause of xeroderma pigmentosum complementation group B (XP-B); also known as xeroderma pigmentosum II (XP2) or XP group B (XPB) or xeroderma pigmentosum group B combined with Cockayne syndrome (XP-B/CS) [:, 4, 5]. Defects in ERCC3 are also a cause of trichothiodystrophy photosensitive (TTDP) [6].

1. Reconstitution of the transcription factor TFIIH: assignment of functions for the three enzymatic subunits, XPB, XPD, and cdk7. Mol. Cell 3, 87-95
2. A history of TFIIH: two decades of molecular biology on a pivotal transcription/repair factor. DNA Repair (Amst.) 10, 714-21
3. Mediator influences Schizosaccharomyces pombe RNA polymerase II-dependent transcription in vitro. J. Biol. Chem. 278, 51301-6
4. Clinical heterogeneity within xeroderma pigmentosum associated with mutations in the DNA repair and transcription gene ERCC3. Am. J. Hum. Genet. 54, 191-200
5. Phenotypic heterogeneity in the XPB DNA helicase gene (ERCC3): xeroderma pigmentosum without and with Cockayne syndrome. Hum. Mutat. 27, 1092-103
6. A mutation in the XPB/ERCC3 DNA repair transcription gene, associated with trichothiodystrophy. Am. J. Hum. Genet. 60, 320-9

This entry represents the helicase Ercc3 (also known as RepB/XPB/Rad25/Ssl2/haywire). Ercc3 is a
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