title: Is Fanconi anemia caused by a defect in the processing of DNA damage?
abstract: Fanconi anemia (FA) is an autosomal genetic disease characterized by a complex array of developmental disorders, a high predisposition to bone marrow failure and to acute myelogenous leukemia. The chromosomal instability and the hypersensitivity to DNA cross-linking agents led to its classification with the DNA repair disorders. This review aimed at establishing whether it is still appropriate to consider 1/approximately FA within a DNA repair framework taking into account the recently discovered genetic heterogeneity characteristics of the defect (eight complementation groups). We discuss the possibility that the FA proteins interact to form a complex which may control different functions, including the processing of specific DNA lesions. Such a complex may act as a sensor to initiate protective systems as well as transcription of specific genes specifying, among others proteins, growth factors. Such steps may be organized as a linear cascade or more likely under the form of a web network.
authors.affiliation: UMR 218 CNRS and LCR no. 1 CEA, Institut Curie-Recherche, Paris, France.
authors.forename: M
authors.initials: M
authors.lastname: Buchwald
authors.forename: E
authors.initials: E
authors.lastname: Moustacchi
mesh_terms: D017209:Apoptosis; D002453:Cell Cycle / Q000502:physiology; D002875:Chromosomes / Q000187:drug effects; D004260:DNA Repair; D005199:Fanconi Anemia / Q000235:genetics* / Q000503:physiopathology; D005816:Genetic Complementation Test; D006133:Growth Substances / Q000502:physiology; D006801:Humans; D016296:Mutagenesis; D017382:Reactive Oxygen Species / Q000502:physiology
chemical_list: D006133:Growth Substances; D017382:Reactive Oxygen Species