The Fanconi anemia pathway: insights from somatic cell genetics using DT40 cell line.
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.
Takata
Minoru
M
Laboratory of DNA Damage Signaling, Department of Late Effect Studies, Radiation Biology Center, Kyoto University, Yoshida-konoe, Sakyo-ku, Kyoto 606-8501, Japan. mtakata@house.rbc.kyoto-u.ac.jp
Ishiai
Masamichi
M
Kitao
Hiroyuki
H
D000818:Animals; D002460:Cell Line; D002645:Chickens; D004249:DNA Damage; D004260:DNA Repair; D004843:Epistasis, Genetic; D005199:Fanconi Anemia / Q000235:genetics* / Q000378:metabolism; D052236:Fanconi Anemia Complementation Group D2 Protein / Q000378:metabolism*; D055786:Gene Knockout Techniques; D009154:Mutation; D010766:Phosphorylation; D011995:Recombination, Genetic; D015398:Signal Transduction; D012854:Sister Chromatid Exchange; D054875:Ubiquitination
D052236:Fanconi Anemia Complementation Group D2 Protein