FRDA is a genetic disorder, due to the abnormal expansion of the GAA trinucleotide repeat in the first intron of the FXN gene, leading to a severe protein deficiency in cells [1,5]; moreover, a small percentage of patients (~4%) are compound heterozygous for the expansion of one FXN allele and for a point mutation on the other, which has deleterious effects on both protein stability and functionality [6,7]. The gene discussed is FXN; the disease is protein S deficiency.