Fanconi anemia: A disorder defective in the DNA damage response

Hiroyuki Kitao, Minoru Takata

Research output: Contribution to journalReview articlepeer-review

62 Citations (Scopus)


Fanconi anemia (FA) is a cancer predisposition disorder characterized by progressive bone marrow failure, congenital developmental defects, chromosomal abnormalities, and cellular hypersensitivity to DNA interstrand crosslink (ICL) agents. So far mutations in 14 FANC genes were identified in FA or FA-like patients. These gene products constitute a common ubiquitin-phosphorylation network called the "FA pathway" and cooperate with other proteins involved in DNA repair and cell cycle control to repair ICL lesions and to maintain genome stability. In this review, we summarize recent exciting discoveries that have expanded our view of the molecular mechanisms operating in DNA repair and DNA damage signaling.

Original languageEnglish
Pages (from-to)417-424
Number of pages8
JournalInternational journal of hematology
Issue number4
Publication statusPublished - Apr 2011

All Science Journal Classification (ASJC) codes

  • Hematology


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