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Mutat Res. 1998 Aug 7;408(2):75-90.

Is Fanconi anemia caused by a defect in the processing of DNA damage?

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  • 1UMR 218 CNRS and LCR no. 1 CEA, Institut Curie-Recherche, Paris, France.

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.

PMID:
9739810
[PubMed - indexed for MEDLINE]
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