Transcription factor II H

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general transcription factor IIH, polypeptide 1, 62kDa
Symbol GTF2H1
Alt. symbols BTF2
Entrez 2965
HUGO 4655
OMIM 189972
RefSeq NM_005316
UniProt P32780
Other data
Locus Chr. 11 p15.1-p14
general transcription factor IIH, polypeptide 2, 44kDa
Symbol GTF2H2
Alt. symbols BTF2, TFIIH, BTF2P44, T-BTF2P44
Entrez 2966
HUGO 4656
OMIM 601748
RefSeq NM_001515
UniProt Q13888
Other data
Locus Chr. 5 q12.2-13.3
general transcription factor IIH, polypeptide 3, 34kDa
Symbol GTF2H3
Alt. symbols BTF2, TFIIH
Entrez 2967
HUGO 4657
OMIM 601750
RefSeq NM_001516
UniProt Q13889
Other data
Locus Chr. 12 q24.31

Transcription factor II Human (TFIIH) is one of several general transcription factors that make up the RNA polymerase II preinitiation complex.[1] TFIIH consists of ten subunits, 7 of which (XPD, XPB, p62, p52, p44, p34 and TTDA) form the core complex. The cyclin activating kinase-subcomplex (CDK7, MAT1, and cyclin H) is linked to the core via the XPD protein[2] Two of the subunits, ERCC2/XPD and ERCC3/XPB, have helicase and ATPase activities and help create the transcription bubble. In a test tube these subunits are only required for transcription if the DNA template is not already denatured or if it is supercoiled.

Two other TFIIH subunits, CDK7 and cyclin H, phosphorylate serine amino acids on the RNA polymerase II C-terminal domain and possibly other proteins involved in the cell cycle. Next to a vital function in transcription initiation, TFIIH is also involved in nucleotide excision repair.

It is responsible for giving the 'go' signal which is why it is assembled last.[citation needed]


Mutation in genes ERCC3/XPB, ERCC2/XPD or TTDA cause trichothiodystrophy, a condition characterized by photosensitivity, ichthyosis, brittle hair and nails, intellectual impairment, decreased fertility and/or short stature.[3]

DNA repair[edit]

TFIIH participates in nucleotide excision repair (NER) by opening the DNA double helix after damage is initially recognized. NER is a multi-step pathway that removes a wide range of different damages that distort normal base pairing, including bulky chemical damages and UV-induced damages. Individuals with mutational defects in genes specifying protein components that catalyze the NER pathway, including the TFIIH components, often display features of premature aging[3][4] (see DNA damage theory of aging).


  1. ^ Kim TK, Ebright RH, Reinberg D (2000). "Mechanism of ATP-dependent promoter melting by transcription factor IIH". Science. 288 (5470): 1418–22. doi:10.1126/science.288.5470.1418. PMID 10827951. 
  2. ^ Lee TI, Young RA (2000). "Transcription of eukaryotic protein-coding genes". Annu. Rev. Genet. 34: 77–137. doi:10.1146/annurev.genet.34.1.77. PMID 11092823. 
  3. ^ a b Theil AF, Hoeijmakers JH, Vermeulen W (2014). "TTDA: big impact of a small protein". Exp. Cell Res. 329 (1): 61–8. doi:10.1016/j.yexcr.2014.07.008. PMID 25016283. 
  4. ^ Edifizi D, Schumacher B (2015). "Genome Instability in Development and Aging: Insights from Nucleotide Excision Repair in Humans, Mice, and Worms". Biomolecules. 5 (3): 1855–69. doi:10.3390/biom5031855. PMC 4598778Freely accessible. PMID 26287260. 

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