Cel The study of primary immunodeficiency diseases has provided significant insights into how the immune system works. This notably includes in vivo immune responses to microorganisms, pathogens or opportunistic agents and in vivo control of reactivity to self, in relation to the identification of specific gene mutations. Hence, some of the key factors of the immune system have been unraveled. In this respect, studies in human conditions are essential because (i) they provide truly natural models of the immune system at work and (ii) because there are many known differences between the human and murine immune systems. Detailed investigations of newly described phenotypes and further delineation of known phenotypes which lack associated gene mutations represent as many new models for studying multiple facets of the human immune system. Appropriate in vitro and in vivo experimental models will be designed in order to gain a detailed understanding of newly identified molecules. It is also expected that the elucidation of immune system pathways will have an impact on therapeutic research. This proposal has been generated by a group of scientists with expertise in both fundamental and clinical research and aims at tackling some of these multifaceted questions. These studies will benefit from the well-characterized cohorts of patients with specific primary immunodeficiencies (PIDs) that have been constituted over the years. The project has 3 major subheadings: 1) Primary immunodeficiencies (PIDs) of DNA repair pathways, 2) PIDs of lymphocyte cytotoxicity/cell death pathways; 3) Therapeutics for primary immunodeficiencies. Scientists who take part in the project will address these various topics as a function of their respective fields of expertise. Also, as observed in the past, the unexpected observation of new phenotypes might significantly impact on research orientations, as a function of their potential interest and feasibility of their analysis. Dziedzina nauki natural sciencesbiological sciencesgeneticsDNAmedical and health sciencesbasic medicineimmunologynatural sciencesbiological sciencesgeneticsmutationnatural sciencesbiological sciencesmicrobiology Program(-y) 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) Temat(-y) ERC-AG-LS6 - ERC Advanced Grant - Immunity and infection Zaproszenie do składania wniosków ERC-2009-AdG Zobacz inne projekty w ramach tego zaproszenia System finansowania ERC-AG - ERC Advanced Grant Instytucja przyjmująca INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE Wkład UE € 1 719 600,00 Adres RUE DE TOLBIAC 101 75654 Paris Francja Zobacz na mapie Region Ile-de-France Ile-de-France Paris Rodzaj działalności Research Organisations Kierownik naukowy Alain Fischer (Prof.) Kontakt administracyjny Nicolas Jeanjean (Mr.) Linki Kontakt z organizacją Opens in new window Strona internetowa Opens in new window Koszt całkowity Brak danych Beneficjenci (1) Sortuj alfabetycznie Sortuj według wkładu UE Rozwiń wszystko Zwiń wszystko INSTITUT NATIONAL DE LA SANTE ET DE LA RECHERCHE MEDICALE Francja Wkład UE € 1 719 600,00 Adres RUE DE TOLBIAC 101 75654 Paris Zobacz na mapie Region Ile-de-France Ile-de-France Paris Rodzaj działalności Research Organisations Kierownik naukowy Alain Fischer (Prof.) Kontakt administracyjny Nicolas Jeanjean (Mr.) Linki Kontakt z organizacją Opens in new window Strona internetowa Opens in new window Koszt całkowity Brak danych