Résumé des résultats (Abstract)
(Anglais)
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Mitochondria are at the core of cellular metabolism, hence it is not surprising that mitochondrial dysfunction is a frequent cause of human disease. This proposal establishes a consortium of 51 European research teams from diverse disciplines, to elucidate the pathogenic mechanisms involved in these disorders. This will involve a combined effort to characterize the components of the apparatus of mitochondrial biogenesis (mtDNA replication, transcription, translation, RNA processing, assembly and transport) in key model organisms, to establish and exploit pathogenic models in cell culture and in whole organisms and to develop techniques for manipulating mitochondrial gene expression, aimed at eventual therapy. The consortium will facilitate this research effort by pooling research tools and resources, promoting researcher exchanges and training, and enhancing scientific communication between scientists with complementary expertise.
This Concerted Action aims to elucidate the molecular, cellular and physiological mechanisms of pathogenesis in mitochondrial disease, and thus chart a route to therapy. MitEURO's key research objectives are to define genes and gene productsinvolved in all of the core genetic and metabolic functions of mitochondria, to profile the global changes in gene expression associated with mitochondrialdys function, to develop pathogenic models of mitochondrial disorders, to characterize mitochondrial signalling pathways and to develop novel strategies for manipulating mitochondrial gene expression. MitEURO aims to facilitate this research effort by pooling of research resources, by promoting research erexchanges and other training initiatives to enhance the European skills base in mitochondrial research, and by developing new tools for the analysis and manipulation of mitochondrial function, notably in the area of bioinformatics.
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