ArticleMolecular and cellular biology2007
DNA repair and transcriptional deficiencies caused by mutations in the Drosophila p52 subunit of TFIIH generate developmental defects and chromosome fragility.
Article in Molecular and cellular biology, 2007. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 29 papers.
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Who cites it
29 citing papers in PubMed, 49 citations in OpenAlex.
- RNA polymerase II transcription initiation with and without XPB translocase activity.Communications biology · 2026Review
- Review
- GTF2H4 regulates partial EndMT via NF-κB activation through NCOA3 phosphorylation in ischemic diseases.Innovation (Cambridge (Mass.)) · 2024Article
- Phosphorylation of XPD drives its mitotic role independently of its DNA repair and transcription functions.Science advances · 2022Article
- Article
- spn-A/rad51 mutant exhibits enhanced genomic damage, cell death and low temperature sensitivity in somatic tissues.Chromosoma · 2021Article
- How to limit the speed of a motor: the intricate regulation of the XPB ATPase and translocase in TFIIH.Nucleic acids research · 2020Article
- Disruption of TFIIH activities generates a stress gene expression response and reveals possible new targets against cancer.Open biology · 2020Article
- Review
- RNAi-mediated depletion of the NSL complex subunits leads to abnormal chromosome segregation and defective centrosome duplication in Drosophila mitosis.PLoS genetics · 2019Article
- Article
- The essential and multifunctional TFIIH complex.Protein science : a publication of the Protein Society · 2018Review
- The intricate network between the p34 and p44 subunits is central to the activity of the transcription/DNA repair factor TFIIH.Nucleic acids research · 2017Article
- Analysis of Drosophila p8 and p52 mutants reveals distinct roles for the maintenance of TFIIH stability and male germ cell differentiation.Open biology · 2016Article
- Protein Profiling of Bladder Urothelial Cell Carcinoma.PloS one · 2016Article
- Architecture of the Human and Yeast General Transcription and DNA Repair Factor TFIIH.Molecular cell · 2015Article
- A Drosophila XPD model links cell cycle coordination with neuro-development and suggests links to cancer.Disease models & mechanisms · 2015Article
- Essential functions of iron-requiring proteins in DNA replication, repair and cell cycle control.Protein & cell · 2014Review
- Article
- Trypanosoma brucei harbours a divergent XPB helicase paralogue that is specialized in nucleotide excision repair and conserved among kinetoplastid organisms.Molecular microbiology · 2013Article
Corrections and comments
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Authors and funding
8 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
The transcription and DNA repair factor TFIIH is composed of 10 subunits. Mutations in the XPB, XPD, and p8 subunits are genetically linked to human diseases, including cancer. However, no reports of mutations in other TFIIH subunits have been reported in higher eukaryotes. Here, we analyze at genetic, molecular, and biochemical levels the Drosophila melanogaster p52 (DMP52) subunit of TFIIH. We found that DMP52 is encoded by the gene marionette in Drosophila and that a defective DMP52 produces UV light-sensitive flies and specific phenotypes during development: organisms are smaller than their wild-type siblings and present tumors and chromosomal instability. The human homologue of DMP52 partially rescues some of these phenotypes. Some of the defects observed in the fly caused by mutations in DMP52 generate trichothiodystrophy and cancer-like phenotypes. Biochemical analysis of DMP52 point mutations introduced in human p52 at positions homologous to those of defects in DMP52 destabilize the interaction between p52 and XPB, another TFIIH subunit, thus compromising the assembly of the complex. This study significantly extends the role of p52 in regulating XPB ATPase activity and, consequently, both its transcriptional and nucleotide excision repair functions.
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