Objectif Our genetic material is continually subjected to damage, either from endogenous sources such as reactive oxygen species produced as by-products of oxidative metabolism, from the breakdown of replication forks during cell growth, or by agents in the environment such as ionising radiation or carcinogenic chemicals. To cope with DNA damage, cells employ elaborate and effective repair processes that specifically recognise a wide variety of lesions in DNA. These repair systems are essential for the maintenance of genome integrity. Unfortunately, some individuals are genetically predisposed to crippling diseases or cancers that are the direct result of mutations in genes involved in the DNA damage response. For several years our work has been at the forefront of basic biological research in the area of DNA repair, and in particular we have made significant contributions to the understanding of inheritable diseases such as breast cancer, Fanconi anemia, and the neurodegenerative disease Ataxia with Oculomotor Apraxia-1 (AOA-1). The focus of this ERC proposal is: (i) to define the phenotypic interplay between three inheritable cancer predisposition syndromes, Fanconi anemia, Bloom s syndrome and breast cancers caused by mutation of BRCA2, (ii) to determine the biological role of the newly discovered GEN1 Holliday junction resolvase in homologous recombination and repair, and (iii) to understand the actions of Aprataxin and Senataxin in relation to the inheritable neurodegenerative diseases AOA-1 and AOA-2, respectively. Our studies will provide an improved understanding of basic mechanisms of DNA repair and thereby underpin future therapeutic developments that will help individuals afflicted with these diseases. Champ scientifique natural sciencesbiological sciencesgeneticsDNAmedical and health sciencesclinical medicineoncologybreast cancernatural sciencesbiological sciencesgeneticsmutationmedical and health sciencesclinical medicinehematologynatural sciencesbiological sciencesgeneticsgenomes Programme(s) 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) Thème(s) ERC-AG-LS1 - ERC Advanced Grant - Molecular and Structural Biology and Biochemistry Appel à propositions ERC-2009-AdG Voir d’autres projets de cet appel Régime de financement ERC-AG - ERC Advanced Grant Institution d’accueil THE FRANCIS CRICK INSTITUTE LIMITED Contribution de l’UE € 2 449 091,40 Adresse 1 MIDLAND ROAD NW1 1AT London Royaume-Uni Voir sur la carte Région London Inner London — West Camden and City of London Type d’activité Research Organisations Contact administratif Heather Joanne Woods (Ms.) Chercheur principal Stephen West (Dr.) Liens Contacter l’organisation Opens in new window Site web Opens in new window Coût total Aucune donnée Bénéficiaires (2) Trier par ordre alphabétique Trier par contribution de l’UE Tout développer Tout réduire THE FRANCIS CRICK INSTITUTE LIMITED Royaume-Uni Contribution de l’UE € 2 449 091,40 Adresse 1 MIDLAND ROAD NW1 1AT London Voir sur la carte Région London Inner London — West Camden and City of London Type d’activité Research Organisations Contact administratif Heather Joanne Woods (Ms.) Chercheur principal Stephen West (Dr.) Liens Contacter l’organisation Opens in new window Site web Opens in new window Coût total Aucune donnée CANCER RESEARCH UK LBG Participation terminée Royaume-Uni Contribution de l’UE Aucune donnée Adresse 2 Redman Place E20 1JQ LONDON Voir sur la carte Région London Inner London — West Camden and City of London Type d’activité Research Organisations Contact administratif Holly Elphinstone (Ms.) Liens Contacter l’organisation Opens in new window Site web Opens in new window Coût total Aucune donnée
