Friedreich’s Ataxia (FRDA) is the most prevalent hereditary disease linked to failed iron-sulfur (Fe-S) cluster biosynthesis. FRDA is a human autosomal recessive genetic disorder caused by a trinucleotide expansion within the frataxin gene (FXN), which codes for the frataxin protein, FXN. The trinucleotide repeat results in an under expression of frataxin. This deficiency pathologically presents as mitochondrial iron overload, increased reactive oxygen species production, and a disruption in Fe-S cluster biosynthesis. Combined, these phenotypes are cytotoxic in metabolic tissues including the dorsal root ganglia and cardiomyocytes. FRDA presents in early adolescence as seen by positive ataxia, poor muscle coordination, and dysarthria. The protein frataxin is a key component in the mitochondrial iron-sulfur cluster bioassembly (ISC) pathway, where it serves as a modulator for cysteine desulfurase, and likely iron delivery to the scaffold.