FRDA cells have been shown to be associated with genome instability11 and have an impaired ability to repair damaged nuclear DNA.15 We sought to determine whether the human and mouse FRDA fibroblasts display genome instability by using imaging flow cytometry detection and immunocytochemical detection of γ-H2AX recruitment to DNA DSBs and comparing the numbers of DSB-positive nuclear foci with the number found in non-FRDA control fibroblasts. This evidence concerns the gene H2AX and Friedreich ataxia.