TRANSREACT

TFIIH as a crucial actor in genome expression and repair

Coordinatore	CENTRE EUROPEEN DE RECHERCHE EN BIOLOGIE ET MEDECINE    more Spiacenti, non ci sono informazioni su questo coordinatore. Contattare Fabio per maggiori infomrazioni, grazie.
Nazionalità Coordinatore	France [FR]
Totale costo	1˙997˙000 €
EC contributo	1˙997˙000 €
Programma	FP7-IDEAS-ERC Specific programme: "Ideas" implementing the Seventh Framework Programme of the European Community for research, technological development and demonstration activities (2007 to 2013)
Code Call	ERC-2008-AdG
Funding Scheme	ERC-AG
Anno di inizio	2009
Periodo (anno-mese-giorno)	2009-01-01   -   2013-12-31

 Partecipanti

#	participant	country	role	EC contrib. [€]
1	CENTRE EUROPEEN DE RECHERCHE EN BIOLOGIE ET MEDECINE  Organization address address: Rue Laurent Fries 1 city: ILLKIRCH GRAFFENSTADEN postcode: 67404 contact info Titolo: Dr. Nome: Steve Cognome: Brooks Email: send email Telefono: +33 3 88 65 33 94 Fax: +33 3 88 65 32 03	FR (ILLKIRCH GRAFFENSTADEN)	hostInstitution	1˙997˙000.00
2	CENTRE EUROPEEN DE RECHERCHE EN BIOLOGIE ET MEDECINE  Organization address address: Rue Laurent Fries 1 city: ILLKIRCH GRAFFENSTADEN postcode: 67404 contact info Titolo: Prof. Nome: Jean-Marc Cognome: Egly Email: send email Telefono: 00 333 88 65 34 47 Fax: 00 333 88 65 32 03	FR (ILLKIRCH GRAFFENSTADEN)	hostInstitution	1˙997˙000.00

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 Obiettivo del progetto (Objective)

'Understanding how the genetic information of a cell is retrieved but also protected from insults, is a major challenge facing modern molecular biology. Indeed, one of the most important developments in human genetics over the last decade, has been the realization that diseases such as cancer but also ageing stem from some dis-regulation in the expression and preservation of the genetic information. TFIIH is a multiprotein complex that is essential in transcription and DNA repair. Mutations in some of its subunits are responsible of a UV sensitivity phenotype in yeast and drosophila. In human, this results in the rare DNA repair deficient genetic disorder, xeroderma pigmentosum (XP), which is characterized by photosensitivity and an increased risk of skin cancers. Two further disorders involving mutation in TFIIH subunits, Cockayne syndrome (CS) and trichothiodystrophy (TTD) are also defective in repair of UV damage, but present quite different clinical features such as brittle hair, neurological and developmental retardation, middles sun sensitivity and no susceptibility to solar carcinogenesis. The clinical complexity of these syndromes cannot be explained solely by deficiencies in DNA repair and emerging evidences indicate that it may also result from a dys-regulation of the transcriptional program under the control of hormones. The goal of this proposal is to investigate the DNA repair/transcription disorders involving mutations in TFIIH. This research will not only assists afflicted XP, CS, TTD and normal individuals in prevention and ultimately cures of hormonal dependent diseases and cancer (the clinical point of view), but more generally will provide an improved understanding of the mechanisms that regulate the expression of protein coding genes and the maintenance of genome integrity (the fundamental research point of view).'