Fanconi anaemia (FA) is a rare, predominantly autosomal recessive syndrome (with 
one X-linked form) that results in congenital defects, abnormal haematopoiesis 
and a greatly increased risk of solid tumours in humans. Mutations in at least 
14 different genes have been shown to cause FA, and several of these genes, 
including FANCJ/BRIP1, FANCD1/BRCA2 and FANCN/PALB2, also predispose to breast 
cancer in heterozygote carriers. The FA genes code for proteins that act in 
complexes to coordinate the repair of damaged DNA, and thus the FA repair 
network is intimately linked with hereditary breast cancer. Much remains to be 
learnt about the functions and interactions of the FA proteins and one 
experimental approach involves the generation of mice that are deficient in 
various FA genes. Mouse models for FANCN/PALB2 have recently been generated, 
including one reported in a recent issue of The Journal of Pathology. Given the 
pivotal role of the PALB2 protein, which interacts with both BRCA1 and BRCA2, 
these mice provide valuable insights into the FA phenotype and mechanisms of 
tumourigenesis caused by disruption of the FA protein network.