YG8R mice are the original transgenic humanized animal model of FRDA generated by Dr. Pook that expresses two human FXN transgenes, containing 82 and 190 GAA repeats, in a murine frataxin null background (fxn−/−FXN+) (Al-Mahdawi et al., 2006). This evidence concerns the gene FXN and Friedreich ataxia.