3R coordination by Fanconi anemia proteins.
Fanconi anemia (FA) is a recessive cancer prone syndrome featuring bone marrow failure and hypersensitivity to DNA crosslinks. Nine FA genes have been isolated so far. The biochemical function(s) of the FA proteins remain(s) poorly determined. However, a large consensus exists on the evidence that, to cope with DNA cross-links, a cell needs a functional FA pathway. In this review, we resume current understanding of how the FA pathway works in response to DNA damage and how it is integrated in a complex network of proteins involved in the maintenance of the genetic stability.
Macé
Gaëtane
G
Institut Gustave-Roussy PR2, UPR2169 du CNRS, 39, rue Camille-Desmoulins, 94805 Villejuif cedex, France.
Bogliolo
Massimo
M
Guervilly
Jean-Hugues
JH
Dugas du Villard
Jean Antoine
JA
Rosselli
Filippo
F
D002453:Cell Cycle; D018797:Cell Cycle Proteins / Q000378:metabolism*; D004249:DNA Damage; D004260:DNA Repair; D004261:DNA Replication; D004268:DNA-Binding Proteins / Q000378:metabolism*; D005199:Fanconi Anemia / Q000235:genetics* / Q000378:metabolism; D051856:Fanconi Anemia Complementation Group Proteins; D005786:Gene Expression Regulation; D030342:Genetic Diseases, Inborn / Q000235:genetics; D042822:Genomic Instability; D006801:Humans; D009687:Nuclear Proteins / Q000378:metabolism*; D013577:Syndrome
D018797:Cell Cycle Proteins; D004268:DNA-Binding Proteins; D051856:Fanconi Anemia Complementation Group Proteins; D009687:Nuclear Proteins