[Fanconi anemia--genotoxic stress and senescence of hematopoietic stem cells].
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.
Yamashita
Takayuki
T
Laboratory of Molecular Genetics, The Institute for Molecular and Cellular Regulation, Gunma University.
Oda
Tsukasa
T
Sekimoto
Takayuki
T
D051017:Apoptosis Regulatory Proteins; D024682:BRCA2 Protein / Q000235:genetics; D002471:Cell Transformation, Neoplastic / Q000235:genetics; D016922:Cellular Senescence / Q000235:genetics*; D002869:Chromosome Aberrations; D004249:DNA Damage / Q000235:genetics*; D005199:Fanconi Anemia / Q000235:genetics*; D000076164:Fanconi Anemia Complementation Group N Protein; D006412:Hematopoietic Stem Cells / Q000473:pathology*; D006801:Humans; D009154:Mutation; D009190:Myelodysplastic Syndromes / Q000209:etiology; D009687:Nuclear Proteins / Q000235:genetics; D025521:Tumor Suppressor Proteins / Q000235:genetics
D051017:Apoptosis Regulatory Proteins; C118024:BLID protein, human; D024682:BRCA2 Protein; C551750:BRCA2 protein, human; D000076164:Fanconi Anemia Complementation Group N Protein; D009687:Nuclear Proteins; C512790:PALB2 protein, human; D025521:Tumor Suppressor Proteins