title: Cockayne syndrome B protein regulates the transcriptional program after UV irradiation.
abstract: The phenotype of the human genetic disorder Cockayne syndrome (CS) is not only due to DNA repair defect but also (and perhaps essentially) to a severe transcription initiation defect. After UV irradiation, even undamaged genes are not transcribed in CSB cells. Indeed, neither RNA pol II nor the associated basal transcription factors are recruited to the promoters of the housekeeping genes, around of which histone H4 acetylation is also deficient. Transfection of CSB restores the recruitment process of RNA pol II. On the contrary, the p53-responsive genes do not require CSB and are transcribed in both wild-type and CSB cells upon DNA damage. Altogether, our data highlight the pivotal role of CSB in initiating the transcriptional program of certain genes after UV irradiation, and also may explain some of the complex traits of CS patients.
authors.affiliation: Institut de Génétique et de Biologie Moléculaire et Cellulaire, CNRS/INSERM, Illkirch, CU Strasbourg, France.
authors.forename: Luca
authors.initials: L
authors.lastname: Proietti-De-Santis
authors.forename: Pascal
authors.initials: P
authors.lastname: Drané
authors.forename: Jean-Marc
authors.initials: JM
authors.lastname: Egly
mesh_terms: D003057:Cockayne Syndrome / Q000235:genetics / Q000378:metabolism*; D004247:DNA / Q000528:radiation effects; D004249:DNA Damage; D004265:DNA Helicases / Q000378:metabolism*; D004260:DNA Repair; D045643:DNA Repair Enzymes; D005786:Gene Expression Regulation; D006801:Humans; D000075223:Poly-ADP-Ribose Binding Proteins; D011401:Promoter Regions, Genetic / Q000235:genetics; D012319:RNA Polymerase II / Q000032:analysis / Q000378:metabolism; D018167:Receptors, Calcitriol / Q000378:metabolism; D013762:Tetrahydrofolate Dehydrogenase / Q000235:genetics; D014158:Transcription, Genetic / Q000528:radiation effects*; D016159:Tumor Suppressor Protein p53 / Q000378:metabolism; D014466:Ultraviolet Rays
chemical_list: D000075223:Poly-ADP-Ribose Binding Proteins; D018167:Receptors, Calcitriol; D016159:Tumor Suppressor Protein p53; D004247:DNA; D013762:Tetrahydrofolate Dehydrogenase; D012319:RNA Polymerase II; D004265:DNA Helicases; C078673:ERCC6 protein, human; D045643:DNA Repair Enzymes