Fanconi anemia: A disorder defective in the DNA damage response

Hiroyuki Kitao, Minoru Takata

Research output: Contribution to journalReview article

55 Citations (Scopus)

Abstract

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
Volume93
Issue number4
DOIs
Publication statusPublished - Apr 2011

All Science Journal Classification (ASJC) codes

  • Hematology

Fingerprint Dive into the research topics of 'Fanconi anemia: A disorder defective in the DNA damage response'. Together they form a unique fingerprint.

  • Cite this