Fanconi anemia pathway and its relationship with cancer

Abstract Fanconi Anemia (FA) is a rare inherited hematological disease, caused by mutations in genes involved the DNA interstrand crosslink (ICL) repair. Up to date, 22 have been identified that encode series of functionally associated proteins recognize ICL lesion and mediate activation downstream repair pathway including nucleotide excision repair, translesion synthesis, homologous recombination. The FA strictly regulated complex mechanisms such as ubiquitination, phosphorylation, degradation signals are essential for maintenance genome stability. Here, we summarize discovery history recent advances genes, further discuss role carcinogenesis cancer therapies.