AML1 haploinsufficiency, gene dosage, and the predisposition to acute leukemia

AML1 haploinsufficiency, gene dosage, and the predisposition to acute leukemia
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DOI:
10.1002/(sici)1521-1878(200003)22:3
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发表时间:
2000-03-01
期刊:
影响因子:
4
通讯作者:
Nucifora, G
Nucifora, G
中科院分区:
生物学3区
文献类型:
--
作者:
Barton, K;Nucifora, G

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造血是一个复杂的发育过程,通过这个过程,未分化的多能造血干细胞产生成熟的功能性血细胞。这一过程在很大程度上受到特定转录因子的调节,这些转录因子控制发育序列所必需的基因表达。白血病是这种正常发育过程中断的一种形式,过去几年的研究表明,许多抑制白血病发生的基因对正常造血也是必不可少的。在最近一个有趣的例子中,Song et al. ((1))证明AML 1基因的单倍不足是一种家族性血小板减少症的遗传基础,这种血小板减少症使受影响的个体易于发展为急性髓性白血病。在这里,我们总结了宋的论文和目前的信息,描述了有趣的剂量效应的基因和其他成员的基因家族。(C)John Wiley & Sons,Inc.
Hematopoiesis is the complex developmental process through which undifferentiated, pluripotent, hematopoietic stem cells come to generate mature, functional blood cells. This process is regulated in large part by specific transcription factors that control expression of genes necessary for the developmental sequence. Leukemias represent one form of disruption of this normal developmental process, and studies over the past few years have shown that many of the genes that underlay leukemogenesis are also essential for normal hematopoiesis. In an interesting recent example, Song et al.((1)) demonstrate that haploinsufficiency of the AML1 gene is the genetic basis of a form of familial thrombocytopenia which predisposes the affected individuals to the development of acute myeloid leukemia. Here we summarize Song's paper and current information describing the interesting dosage effects of this gene and other members of its gene family. (C) 2000 John Wiley & Sons, Inc.