Lactic acidosis of Saguenay-Lac-Saint-Jean region, also known as Leigh syndrome, is a genetic disorder that occurs mainly in the Saguenay-Lac-Saint-Jean region. This disease mainly affects the liver and brain. People with lactic acidosis of Saguenay-Lac-Saint-Jean region are likely to suffer from seizures that lead to death in about 80% of them before the age of 4 years. These seizures can be triggered by emotional stress, an infection, and a diet high in fat and sugar. Our work aims to identify and prevent the mechanisms responsible for crises. We use cell and animal models in which we apply therapeutic strategies to help people with lactic acidosis of Saguenay-Lac-Saint-Jean region improve their quality of life.