| Type: | Family | Name: | Helicase Ercc3 |
| Description: | 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 []. 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) []. Errc3 acts by opening DNA either around the RNA transcription start site or the DNA damage []. 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) [:, , ]. Defects in ERCC3 are also a cause of trichothiodystrophy photosensitive (TTDP) []. | Short Name: | Helicase_Ercc3 |
