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Nihon Rinsho. 2008 Mar;66(3):477-82.

[Fanconi anemia--genotoxic stress and senescence of hematopoietic stem cells].

[Article in Japanese]

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Laboratory of Molecular Genetics, The Institute for Molecular and Cellular Regulation, Gunma University.


Fanconi anemia (FA) is a genetically heterogeneous inherited disorder characterized by progressive bone marrow failure, development of hematopoietic and solid malignancies and genomic instability. 13 FA proteins, identified to date, closely cooperate with familial breast cancer susceptibility proteins such as BRCA2 and PALB2, thereby forming 'the FA/BRCA molecular network'. Here I summarize our recent understanding of the molecular network and its significance in the pathogenesis of FA. I emphasize that FA provides an excellent genetic model for studying senescence and malignant transformation of human hematopoietic stem cells.

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