[Fanconi anemia: cellular and molecular features]

  • Macé G
  • Briot D
  • Guervilly J
 et al. 
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Abstract

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.

Author-supplied keywords

  • Bone Marrow Transplantation
  • Chromosomes, Human
  • Chromosomes, Human: drug effects
  • Cross-Linking Reagents
  • Cross-Linking Reagents: adverse effects
  • DNA
  • DNA Damage
  • DNA Repair
  • DNA Repair: genetics
  • DNA: drug effects
  • Fanconi Anemia
  • Fanconi Anemia Complementation Group Proteins
  • Fanconi Anemia Complementation Group Proteins: def
  • Fanconi Anemia Complementation Group Proteins: gen
  • Fanconi Anemia Complementation Group Proteins: phy
  • Fanconi Anemia: diagnosis
  • Fanconi Anemia: genetics
  • Fanconi Anemia: pathology
  • Fanconi Anemia: therapy
  • Gene Therapy
  • Genes, Recessive
  • Genetic Complementation Test
  • Genetic Heterogeneity
  • Hematopoiesis
  • Homeostasis
  • Humans
  • Mutagens
  • Mutagens: adverse effects
  • Oxidative Stress
  • Phenotype
  • Tumor Necrosis Factor-alpha
  • Tumor Necrosis Factor-alpha: physiology

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Authors

  • G Macé

  • D Briot

  • J-H Guervilly

  • F Rosselli

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