The Fanconi anemia pathway and the DNA interstrand cross-links repair.
Fanconi anemia (FA) is a genetic cancer-predisposition syndrome characterized by bone marrow failure and cellular and chromosomal hypersensitivity to DNA cross-linking agents. Seven FA genes have been isolated and their products associate to form a pathway that interacts functionally or physically with several DNA-damage response proteins involved in cell cycle checkpoints and/or DNA repair. These proteins include BLM, ATM, BRCA1, XPF and the MRE11/RAD50/NBS1 complex. In spite of several recent striking progresses in the biochemistry and the molecular biology of the disorder, the precise function(s) of the FA proteins remain(s) poorly determined. However, several recent data indicate that the FA pathway could be involved in the coordination of both cell cycle checkpoints and DNA repair.
Rosselli
Filippo
F
Institut Gustave Roussy PR2, UPR 2169 du CNRS, 39, rue Camille-Desmoulins, 94805 Villejuif, France. rosselli@igr.fr
Briot
Delphine
D
Pichierri
Pietro
P
D000818:Animals; D003432:Cross-Linking Reagents / Q000737:chemistry / Q000378:metabolism*; D004249:DNA Damage; D004260:DNA Repair; D005199:Fanconi Anemia / Q000235:genetics* / Q000503:physiopathology*; D018816:Genes, cdc / Q000502:physiology; D006801:Humans
D003432:Cross-Linking Reagents