RGD Reference Report - A history of TFIIH: two decades of molecular biology on a pivotal transcription/repair factor. - Rat Genome Database

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A history of TFIIH: two decades of molecular biology on a pivotal transcription/repair factor.

Authors: Egly, Jean-Marc  Coin, Frédéric 
Citation: Egly JM and Coin F, DNA Repair (Amst). 2011 Jul 15;10(7):714-21. doi: 10.1016/j.dnarep.2011.04.021. Epub 2011 May 17.
RGD ID: 9681726
Pubmed: PMID:21592869   (View Abstract at PubMed)
DOI: DOI:10.1016/j.dnarep.2011.04.021   (Journal Full-text)

The TFIIH multiprotein complex is organized into a 7-subunit core associated with a 3-subunit CDK-activating kinase module (CAK). Three enzymatic subunits are present in TFIIH, two ATP-dependent DNA helicases: XPB and XPD, and the kinase Cdk7. Mutations in three of the subunits, XPB, XPD and TTDA, lead to three distinct genetic disorders: xeroderma pigmentosum (XP), Cockayne syndrome (CS) and trichothiodystrophy (TTD) predisposing patients not only to cancer and ageing but also to developmental and neurological defects. These heterogeneous phenotypes originate from the dual role of TFIIH in transcription and DNA repair. For twenty years, many molecular studies have been conducted with the aim to unveil the role of TFIIH in DNA repair and transcription as well as the origin of the phenotypes of patients. This review intends to give a non-exhaustive survey of the most prominent discoveries on the molecular functioning of TFIIH.

Molecular Pathway Annotations    Click to see Annotation Detail View
Objects Annotated

Genes (Rattus norvegicus)
Ccnh  (cyclin H)
Cdk7  (cyclin-dependent kinase 7)
Ercc2  (ERCC excision repair 2, TFIIH core complex helicase subunit)
Ercc3  (ERCC excision repair 3, TFIIH core complex helicase subunit)
Ercc5  (ERCC excision repair 5, endonuclease)
Gtf2h1  (general transcription factor IIH subunit 1)
Gtf2h2  (general transcription factor IIH subunit 2)
Gtf2h3  (general transcription factor IIH subunit 3)
Gtf2h4  (general transcription factor 2H subunit 4)
Gtf2h5  (general transcription factor IIH subunit 5)
Mnat1  (MNAT1 component of CDK activating kinase)

Genes (Mus musculus)
Ccnh  (cyclin H)
Cdk7  (cyclin dependent kinase 7)
Ercc2  (excision repair cross-complementing rodent repair deficiency, complementation group 2)
Ercc3  (excision repair cross-complementing rodent repair deficiency, complementation group 3)
Ercc5  (excision repair cross-complementing rodent repair deficiency, complementation group 5)
Gtf2h1  (general transcription factor II H, polypeptide 1)
Gtf2h2  (general transcription factor II H, polypeptide 2)
Gtf2h3  (general transcription factor IIH, polypeptide 3)
Gtf2h4  (general transcription factor II H, polypeptide 4)
Gtf2h5  (general transcription factor IIH, polypeptide 5)
Mnat1  (menage a trois 1)

Genes (Homo sapiens)
CCNH  (cyclin H)
CDK7  (cyclin dependent kinase 7)
ERCC2  (ERCC excision repair 2, TFIIH core complex helicase subunit)
ERCC3  (ERCC excision repair 3, TFIIH core complex helicase subunit)
ERCC5  (ERCC excision repair 5, endonuclease)
GTF2H1  (general transcription factor IIH subunit 1)
GTF2H2  (general transcription factor IIH subunit 2)
GTF2H3  (general transcription factor IIH subunit 3)
GTF2H4  (general transcription factor IIH subunit 4)
GTF2H5  (general transcription factor IIH subunit 5)
MNAT1  (MNAT1 component of CDK activating kinase)


Additional Information