One murine model based on conditional ablation of mouse Fxn results in complete knockout of Fxn in cardiac and skeletal muscle [23]; although this model presents an aggressive FRDA-like phenotype, including cardiomyopathy and mitochondrial defects, it does not mirror the human genotype of trinucleotide repeats, which partially silence expression of the otherwise fully functional protein. The gene discussed is FXN; the disease is cardiomyopathy.