Objectif Develop transgenic mice with different mutation in the FMR1 gene.Study the biochemical abnormalities of these transgenic mice.Morphological, cellular and molecular studies of the brain in the mice.Studies of behaviour and cognitive function in these transgenic animals. To study the mechanisms that lead to mental retardation and behavioral abnormalities in the fragile X syndrome, we take advantage by the participants' expertise in molecular biology, neurobiology and knowledge of animal behavior. Fragile X mental retardation is caused by the absence of the FMR1 protein or by in missense mutation in the FMR1 protein. For this purpose we will engineer mice that have abnormalities in the FMR1 gene that will mimic the effect at the protein level as is seen in patient suffering from fragile X mental retardation. Animals will be prepared that produce no protein or a mutated FMR1 protein. By reintroduction of the gene in these animals we will bring the gene to expression into a limited number of tissues and/or cell types. These animals will be used to study clinical and biochemical abnormalities. These mice will be studied morphologically, electrophysiologically and molecularly. As fragile X patients show deficits in a number of behavior and cognitive functions, these mice will be subjected to a number of behavior or cognitive tests to determine which functions are affected and to try to understand which parts of the brain are important for the development of mental retardation. The studies on the transgenic mice might contribute to our understanding of behavior, learning and memory. Champ scientifique natural sciencesbiological sciencesneurobiologycognitive neurosciencenatural sciencesbiological sciencesbiochemistrybiomoleculesproteinsnatural sciencesbiological sciencesgeneticsmutationnatural sciencesbiological sciencesbiological behavioural sciencesethologynatural sciencesbiological sciencesmolecular biology Programme(s) FP4-BIOMED 2 - Specific research, technological development and demonstration programme in the field of biomedicine and health, 1994-1998 Thème(s) 3.4 - Genetic and immunological basis of neuromental diseases Appel à propositions Data not available Régime de financement CSC - Cost-sharing contracts Coordinateur Erasmus Universiteit Rotterdam Contribution de l’UE Aucune donnée Adresse 50,Dr. Molewaterplein 3000 DR Rotterdam Pays-Bas Voir sur la carte Coût total Aucune donnée Participants (3) Trier par ordre alphabétique Trier par contribution de l’UE Tout développer Tout réduire Born-Bunge Foundation Belgique Contribution de l’UE Aucune donnée Adresse 1,Universiteitsplein 2610 Wilrijk Voir sur la carte Coût total Aucune donnée Institut National de la Santé et de la Recherche Médicale France Contribution de l’UE Aucune donnée Adresse 123,Boulevard de Port-Royal 75674 Paris Voir sur la carte Coût total Aucune donnée UNIVERSITAIRE INSTELLING ANTWERPEN - UNIVERSITEIT ANTWERPEN Belgique Contribution de l’UE Aucune donnée Adresse 1,Universiteitsplein 1 2610 WILRIJK (ANTWERPEN) Voir sur la carte Coût total Aucune donnée