Mitochondrie et maladies neurodégénératives

Neurons are highly differentiated cells, specialized for intercellular communication. Their physiological functions require high energy levels. They are particularly sensitive to metabolic stress. The importance of mitochondria in neurons is sustained by the frequent neurological phenotype in mitochondrial diseases. Mitochondria are involved in many physiological processes ranging from ATP energy production, lipid and amino-acid metabolism, calcium homeostasis, thermogenesis, reactive oxygen species production, axonal transport and apoptosis. Disruption of each of these processes may contribute to neurodegeneration. Last year, mitochondrial dysfunction has been related to neurodegenerative disorders such as Alzheimer's disease, Parkinson's disease, Huntington's disease and amyotrophic lateral sclerosis. Thus, better understanding the pathophysiologic mechanisms allows to consider new therapeutic approaches.