First, BRCA1 is post-transcriptionally down-regulated by p210[15]; second, while BRCA1 is not essential for FANCD2 monoubiquitination[28] it is required for FANCD2 binding to γH2AX at stalled replication forks[29] and for the subsequent formation of FANCD2 foci after DNA damage[27], [28]; and third, BRCA1−/− cells share with FA cells a chromosomal instability phenotype[45]. The gene discussed is BRCA1; the disease is Friedreich ataxia.