Fanconi anemia (FA) is a chromosomal instability syndrome characterized by the 
presence of pancytopenia, congenital malformations and cancer predisposition. 
Six genes associated with this disorder have been cloned, and mice with targeted 
disruptions of several of the FA genes have been generated. These mouse models 
display the characteristic FA feature of cellular hypersensitivity to DNA 
cross-linking agents. Although they do not develop hematological or 
developmental abnormalities spontaneously, they mimic FA patients in their 
reduced fertility. Studies using these animal models provide valuable insights 
into the involvement of apoptotic pathways in FA, and help characterize the 
defects in FA hematopoietic cells. In addition, mouse models are also useful for 
testing treatments for FA.