Strikingly, the L607H mutation in MLH1 observed in hereditary nonpolyposis colon cancer (HNPCC) disrupts the interaction with FANCJ (Xie et al. 2010a), which raises the possibility that the FANCJ–MLH1 interaction is important for preventing colorectal cancers. The gene discussed is BRIP1; the disease is hereditary nonpolyposis colon cancer.