Dr. Pierre Rustin is interested in the understanding and the curing of mitochondrial diseases, in particular those linked to respiratory chain deficiency. Respiratory chain defects can lead to superoxide overproduction and cardiomyopathy as in diseases linked to complex I deficiency and Friedreich ataxia. The specific know-how of the group is our ability through biochemical approaches (spectrophotometry and polarography) to detect mild defects of the respiratory chain and Krebs cycle. As it has been shown that oxidative stress is widely involved in cardiovascular disease, we also focus our attention on the pathways regulating the response to oxidative stress insult. We study different models ranging from patients fibroblasts to mouse models with the objective to propose therapies.