The Fanconi anemia/BRCA pathway: a coordinator of cross-link repair.
Fanconi anemia (FA) is a rare inherited disease characterized by genomic instability and markedly increased cancer risk. Efforts to elucidate the molecular basis of FA have unearthed a novel DNA damage response pathway, the integrity of which is critical for cellular resistance to DNA cross-linking agents. Despite significant progress in uncovering the molecular events underlying FA, the precise function of this pathway in DNA repair is unknown. This article will review evidence implicating FA proteins in multiple aspects of DNA cross-link repair and propose a model to explain the selectivity of the FA pathway toward DNA cross-linking agents.
Mirchandani
Kanchan D
KD
Department of Radiation Oncology, Dana-Farber Cancer Institute, Harvard Medical School, Boston, MA 02115, USA.
D'Andrea
Alan D
AD
D000818:Animals; D003432:Cross-Linking Reagents / Q000494:pharmacology; D004260:DNA Repair; D005199:Fanconi Anemia / Q000235:genetics*; D006801:Humans; D008957:Models, Genetic; D009687:Nuclear Proteins / Q000502:physiology; D011995:Recombination, Genetic
D003432:Cross-Linking Reagents; D009687:Nuclear Proteins