FANCF, as an adaptor protein among 14 FA complementation (FANC) groups (FA-A, -B, -C, -D1, -D2, -E, -F, -G, -I, -J, -L, -M, -N and -P) and one FA-like complementation group (FA-O), is critically involved in regulating the function of the FA/BRCA pathway by maintaining the stability of the FA core complex and ubiquitin activation (monoubiquitination) of the FANCD2 protein (7–9). The gene discussed is FANCF; the disease is Friedreich ataxia.