HDACi of the 2-amino-benzamide class, such as pimelic diphenylamide derivative (11), which selectively target HDAC3, have been shown to decondense the chromatin at the FXN locus and restore frataxin levels in Friendeich’s ataxia (FRDA) patient-derived cells and in mouse models of the disease, offering a promising approach for treating Friedreich’s ataxia [65,74,75]. Here, HDAC3 is linked to Friedreich ataxia.