The Fanconi anemia pathway: insights from somatic cell genetics using DT40 cell line

Mutat Res. 2009 Jul 31;668(1-2):92-102. doi: 10.1016/j.mrfmmm.2008.12.012. Epub 2009 Jan 17.

Abstract

The Fanconi anemia (FA) pathway is a complex phosphorylation-ubiquitination network in the DNA damage signaling, which is still poorly understood. Defects in the "FA pathway" or in the related DNA repair proteins cause FA, a hereditary disorder that accompanies compromised DNA crosslink repair, poor hematopoetic stem cell survival, genomic instability, and cancer. For molecular dissection of the FA pathway, we have been using chicken B cell line DT40 as a model system. In this review, we will summarize our current understanding of the pathway, and discuss how studies using DT40 have contributed to this rapidly evolving field.

Publication types

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

MeSH terms

  • Animals
  • Cell Line
  • Chickens
  • DNA Damage
  • DNA Repair*
  • Epistasis, Genetic
  • Fanconi Anemia / genetics*
  • Fanconi Anemia / metabolism
  • Fanconi Anemia Complementation Group D2 Protein / metabolism*
  • Gene Knockout Techniques
  • Mutation
  • Phosphorylation
  • Recombination, Genetic
  • Signal Transduction
  • Sister Chromatid Exchange
  • Ubiquitination

Substances

  • Fanconi Anemia Complementation Group D2 Protein