Emergence of a DNA-damage response network consisting of Fanconi anaemia and BRCA proteins

Nat Rev Genet. 2007 Oct;8(10):735-48. doi: 10.1038/nrg2159. Epub 2007 Sep 4.

Abstract

Fanconi anaemia (FA) has recently become an attractive model to study breast cancer susceptibility (BRCA) genes, as three FA genes, FANCD1, FANCN and FANCJ, are identical to the BRCA genes BRCA2, PALB2 and BRIP1. Increasing evidence shows that FA proteins function as signal transducers and DNA-processing molecules in a DNA-damage response network. This network consists of many proteins that maintain genome integrity, including ataxia telangiectasia and Rad3 related protein (ATR), Bloom syndrome protein (BLM), and BRCA1. Now that the gene that is defective in the thirteenth and last assigned FA complementation group (FANCI) has been identified, I discuss what is known about FA proteins and their interactive network, and what remains to be discovered.

Publication types

  • Research Support, N.I.H., Intramural
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Animals
  • BRCA2 Protein / genetics
  • BRCA2 Protein / metabolism
  • Chromatin / metabolism
  • DNA / metabolism
  • DNA Damage* / genetics
  • DNA Repair*
  • DNA-Binding Proteins / genetics
  • DNA-Binding Proteins / metabolism
  • Fanconi Anemia / genetics*
  • Fanconi Anemia / metabolism*
  • Fanconi Anemia Complementation Group N Protein
  • Fanconi Anemia Complementation Group Proteins / metabolism*
  • Humans
  • Nuclear Proteins / genetics
  • Nuclear Proteins / metabolism
  • RNA Helicases / genetics
  • RNA Helicases / metabolism
  • Signal Transduction*
  • Tumor Suppressor Proteins / genetics
  • Tumor Suppressor Proteins / metabolism
  • Ubiquitin-Protein Ligases / metabolism

Substances

  • BRCA2 Protein
  • Chromatin
  • DNA-Binding Proteins
  • Fanconi Anemia Complementation Group N Protein
  • Fanconi Anemia Complementation Group Proteins
  • Nuclear Proteins
  • PALB2 protein, human
  • Tumor Suppressor Proteins
  • DNA
  • Ubiquitin-Protein Ligases
  • BRIP1 protein, human
  • RNA Helicases