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Mitochondrial functionality and fuel dependency analysis Fibroblasts were firstly seeded in Seahorse XF Cell Culture Microplates at a density of 2 × 10 4 cells per well for unrelated controls and 4 × 10 4 cells per well for familial control and mutation carriers (both asymptomatic and symptomatic) in 180 μL of growth medium, then allowed to adhere for 1 h at room temperature and finally incubated for 24 h in a 37 °C humidified incubator with 5% CO 2