3R coordination by Fanconi anemia proteins

Biochimie. 2005 Jul;87(7):647-58. doi: 10.1016/j.biochi.2005.05.003.

Abstract

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.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Cell Cycle
  • Cell Cycle Proteins / metabolism*
  • DNA Damage*
  • DNA Repair
  • DNA Replication
  • DNA-Binding Proteins / metabolism*
  • Fanconi Anemia / genetics*
  • Fanconi Anemia / metabolism
  • Fanconi Anemia Complementation Group Proteins
  • Gene Expression Regulation
  • Genetic Diseases, Inborn / genetics
  • Genomic Instability
  • Humans
  • Nuclear Proteins / metabolism*
  • Syndrome

Substances

  • Cell Cycle Proteins
  • DNA-Binding Proteins
  • Fanconi Anemia Complementation Group Proteins
  • Nuclear Proteins