Molecular Pathogenesis of Fanconi Anemia: Current Understanding.
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概要
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Fanconi anemia (FA) is an autosomal recessive disease, characterized by bone marrow failure, leukemia susceptibility and chromosome instability. There are at least eight genetically distinct groups (A, B, C, D1, D2, E, F and G). To date, seven genes (for FA-A, C, D1, D2, E, F and G) have been cloned. These FA proteins cooperate in a common molecular pathway. FANCD2, a downstream component of this pathway, interacts with BRCA1, a familial breast cancer gene product. These findings, together with recent identification of BRCA2 as the FANCD1 gene, suggest that the FA/BRCA pathway regulates homology-directed DNA repair. The present review describes a current view on the FA/BRCA pathway and highlights the genetics of FA. The FA/BRCA pathway plays an important role in the maintenance of genomic stability in hematopoietic cells. Elucidation of the molecular mechanisms of this pathway would provide new insights into the pathogenesis of acquired bone marrow failure syndromes and myeloid malignancies.
- 特定非営利活動法人 日本小児血液・がん学会の論文
特定非営利活動法人 日本小児血液・がん学会 | 論文
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