The emerging genetic and molecular basis of Fanconi anaemia

The past few years have witnessed a considerable expansion in our understan ding of the pathways that maintain chromosome stability in dividing cells t hrough the identification of genes that are mutated in certain human chromo some instability disorders. Cells that are derived from patients with Fanco ni anaemia (FA) show spontaneous chromosomal instability and mutagen hypers ensitivity, but FA poses a unique challenge as the nature of the DNA damage -response pathway thought to be affected by the disease has long been a mys tery. However, the recent cloning of most of the FA-associated genes, and t he characterization of their protein products, has provided tantalizing clu es as to the molecular basis of this disease.