Mutations in more than 20 genes are known to cause FA and encode proteins primarily involved in DNA repair and HR that constitute the FANC/BRCA repair pathway, which inactivation results in chromosomal fragility and cellular hypersensitivity to DNA-damaging agents, particularly those inducing cross-links between DNA strands, such as mitomycin C (MMC) or inhibiting PARP1 activity [12]. Here, PARP1 is linked to Friedreich ataxia.