Increased activity and expression of ER stress markers such as the activating transcription factor 4 (ATF4), the chaperone binding immunoglobulin protein (BiP) and phosphorylated eIF2α have been found in cellular (Cortopassi et al., 2006; Lu and Cortopassi, 2007) and mouse (Huang et al., 2013) FRDA models. Here, ATF4 is linked to Friedreich ataxia.