In contrast to what has been observed in mice, humans heterozygous for pathogenicBRCA1, BRCA2 and PALB2 germline mutations arepredisposed to several tumors (Antoniou etal., 2003, 2014; Liu et al., 2012; Roy et al., 2012), andbiallelic mutations in these genes result in FA (Howlett et al., 2002; Reid et al., 2007; Sawyer et al., 2015). The gene discussed is PALB2; the disease is Friedreich ataxia.