CKM and cardiomyopathy: Further evidence for the role of ROS in FRDA pathogenesis provided beneficial effects of antioxidants like idebenone on the cardiac symptoms of FRDA patients and in a murine FRDA cardiomyopathy (Frda/MCK) model (Hausse et al., 2002; Mariotti et al., 2003; Seznec et al., 2004).