Fanconi anemia: A disorder defective in the DNA damage response

Hiroyuki Kitao, Minoru Takata

研究成果: Contribution to journalReview article査読

58 被引用数 (Scopus)

抄録

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.

本文言語英語
ページ(範囲)417-424
ページ数8
ジャーナルInternational journal of hematology
93
4
DOI
出版ステータス出版済み - 4 2011

All Science Journal Classification (ASJC) codes

  • Hematology

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