Discovered 13 years ago as a BRCA1-interacting protein, FANCJ helicase is now regarded as a bona fide tumor suppressor and genetically linked to the cancer-prone disorder FA characterized by progressive bone marrow failure; however, the precise function(s) of FANCJ in the FA pathway of ICL repair is still not well understood. The gene discussed is BRIP1; the disease is Friedreich ataxia.