[Fanconi anemia: cellular and molecular features].
Fanconi anemia (FA) is a recessive human cancer prone syndrome featuring bone marrow failure, developmental abnormalities and hypersensitivity to DNA crosslinking agents exposure. 11 among 12 FA gene have been isolated. The biochemical functions of the FANC proteins remain poorly understood. Anyhow, to cope with DNA crosslinks a cell needs a functional FANC pathway. Moreover, the FANC proteins appear to be involved in cell protection against oxidative damage and in the control of TNF-alpha activity. In this review, we describe the current understanding of the FANC pathway and we present how it may be integrated in the complex networks of proteins involved in maintaining the cellular homeostasis.
Macé
G
G
Equipe Voie FANC/BRCA et Cancer FRE 2939 CNRS, Institut Gustave-Roussy, 39, rue Camille-Desmoulins, 94805 Villejuif, France.
Briot
D
D
Guervilly
J-H
JH
Rosselli
F
F
D016026:Bone Marrow Transplantation; D002877:Chromosomes, Human / Q000187:drug effects; D003432:Cross-Linking Reagents / Q000009:adverse effects; D004247:DNA / Q000187:drug effects; D004249:DNA Damage; D004260:DNA Repair / Q000235:genetics; D005199:Fanconi Anemia / Q000175:diagnosis / Q000235:genetics / Q000473:pathology* / Q000628:therapy; D051856:Fanconi Anemia Complementation Group Proteins / Q000172:deficiency / Q000235:genetics* / Q000502:physiology; D005808:Genes, Recessive; D005816:Genetic Complementation Test; D018740:Genetic Heterogeneity; D015316:Genetic Therapy; D006410:Hematopoiesis; D006706:Homeostasis; D006801:Humans; D009153:Mutagens / Q000009:adverse effects; D018384:Oxidative Stress; D010641:Phenotype; D014409:Tumor Necrosis Factor-alpha / Q000502:physiology
D003432:Cross-Linking Reagents; D051856:Fanconi Anemia Complementation Group Proteins; D009153:Mutagens; D014409:Tumor Necrosis Factor-alpha; D004247:DNA