FXN and Friedreich ataxia: Furthermore, in a mouse model of Friedreich’s Ataxia (FRDA), an autosomal recessive disease marked by substantially reduced levels of the mitochondrial ISCU regulatory protein frataxin (Ftx) [250,251], the absence of Ftx function resulted in Fe-S protein deficiency, mitochondrial iron accumulation, and increased LD abundance in cardiac muscle cells [252].