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International Journal of Hematology
Published in: International Journal of Hematology 4/2011

01-04-2011 | Progress in Hematology

Fanconi anemia: a disorder defective in the DNA damage response

Authors: Hiroyuki Kitao, Minoru Takata

Published in: International Journal of Hematology | Issue 4/2011

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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.
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Metadata
Title
Fanconi anemia: a disorder defective in the DNA damage response
Authors
Hiroyuki Kitao
Minoru Takata
Publication date
01-04-2011
Publisher
Springer Japan
Published in
International Journal of Hematology / Issue 4/2011
Print ISSN: 0925-5710
Electronic ISSN: 1865-3774
DOI
https://doi.org/10.1007/s12185-011-0777-z

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