In the present study, we described for the first time that CCN2 deficiency protected from FA-AKI by decreasing tubular cell injury, as shown by milder tubular dilatation, cast formation, loss of tubular brush border, and KIM-1 protein expression, a proposed biomarker for renal injury [55,56], as well as decreasing cell death. This evidence concerns the gene CCN2 and Friedreich ataxia.