This group investigates, in the context of neurogenetic diseases, the processes of neuronal dysfunction and death in order to find ways of stimulating neuronal survival and repair, with a view to their possible therapeutic application. Within the broad spectrum of neurodegenerative diseases, we have focused on those affecting the cerebellum and spinal cord, with an emphasis on Friedreich's ataxia, a neurogenetic disease caused by a deficiency of frataxin, a mitochondrial protein encoded in the nuclear genome.