Thus, this work aims to deeply investigate whether the mitochondrial metabolism alterations observed in FA depend on altered expression of proteins belonging to the oxidative phosphorylation (OxPhos) machinery or mitochondrial biogenesis and dynamics modulators in lymphoblasts and fibroblasts carrying the mutated FANC-A gene, comparing the results with isogenic-corrected FANC-A gene cell lines. The gene discussed is FANCA; the disease is Friedreich ataxia.