Fanconi anemia group A protein (FANCA) is a DNA repair protein that operates in a postreplication repair or a cell cycle checkpoint function. FANCA is involved in interstrand DNA cross-link repair and in the maintenance of normal chromosome stability. Defects in FANCA are the cause of Fanconi anemia, a syndrome characterized by pancytopenia, congenital malformations and predisposition to cancers caused by a hypersensitivity to DNA-damaging agents, chromosomal instability and defective DNA repair.