Komeno Yukiko, Kitaura Jiro, Kitamura Toshio
Division of Cellular Therapy, Institute of Medical Science, the University of Tokyo, Tokyo, Japan.
J Cell Physiol. 2009 Jun;219(3):529-34. doi: 10.1002/jcp.21739.
Myelodysplastic syndrome (MDS) is a clonal disorder of hematopietic stem cells characterized by ineffective hematopoiesis, peripheral blood cytopenia, morphologic dysplasia, and susceptibility to acute myeloid leukemia. Several mechanisms have been suggested as causes of MDS: unbalanced chromosomal abnormalities reflecting a gain or loss of chromosomal material, point mutations of transcription factors, and inactivation of p53. However, appropriate animal models that mimic MDS have long been lacking. We recently reported a novel murine model of MDS that recapitulates trilineage dysplasia and transformation to AML. In this review, we summarize the animal models of MDS and discuss the molecular bases of MDS as well as those of leukemia and myeloproliferative disorders (MPD). J. Cell. Physiol. 219: 529-534, 2009. (c) 2009 Wiley-Liss, Inc.
骨髓增生异常综合征（MDS）是一种造血干细胞的克隆性疾病，其特征为无效造血、外周血细胞减少、形态学发育异常以及易患急性髓系白血病。已有多种机制被认为是MDS的病因：反映染色体物质增减的不平衡染色体异常、转录因子的点突变以及p53的失活。然而，长期以来一直缺乏能够模拟MDS的合适动物模型。我们最近报道了一种新型的MDS小鼠模型，该模型概括了三系发育异常并转化为急性髓系白血病。在这篇综述中，我们总结了MDS的动物模型，并讨论了MDS以及白血病和骨髓增殖性疾病（MPD）的分子基础。《细胞生理学杂志》219: 529 - 534, 2009。（c）2009威利 - 利斯公司。
