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造血细胞中Nf1的体细胞失活会导致进行性骨髓增殖性疾病。

Somatic inactivation of Nf1 in hematopoietic cells results in a progressive myeloproliferative disorder.

作者信息

Le Doan T, Kong Namie, Zhu Yuan, Lauchle Jennifer O, Aiyigari Abigail, Braun Benjamin S, Wang Endi, Kogan Scott C, Le Beau Michelle M, Parada Luis, Shannon Kevin M

机构信息

Department of Pediatrics, University of California, San Francisco, 513 Parnassus Ave, HSE 302, San Francisco, CA 94143, USA.

出版信息

Blood. 2004 Jun 1;103(11):4243-50. doi: 10.1182/blood-2003-08-2650. Epub 2004 Feb 24.

Abstract

The NF1 tumor suppressor gene encodes a guanosine triphosphotase (GTPase)-activating protein that negatively regulates Ras signaling and is inactivated in a subset of juvenile myelomonocytic leukemias (JMMLs). Adoptive transfer of fetal liver cells from Nf1 mutant mice models JMML; however, this system has important limitations as a platform for performing biologic and preclinical studies. We have exploited the interferon-inducible Mx1-Cre transgene to ablate a conditional mutant Nf1 allele in hematopoietic cells. Somatic inactivation of Nf1 induces a myeloproliferative disorder with 100% penetrance that is associated with a sub-acute clinical course, tissue infiltration by myeloid cells, hypersensitivity to granulocyte-macrophage colony stimulating factor, hyperproliferation, and resistance to apoptosis. These Mx1-Cre, Nf1(flox/flox) mice establish a tractable experimental model for testing therapeutics and for identifying mutations that cooperate with hyperactive Ras in myeloid leukemogenesis.

摘要

神经纤维瘤病1型(NF1)肿瘤抑制基因编码一种鸟苷三磷酸酶(GTPase)激活蛋白,该蛋白对Ras信号传导起负调节作用,并且在一部分青少年型粒单核细胞白血病(JMML)中失活。将Nf1突变小鼠的胎肝细胞进行过继性移植可模拟JMML;然而,作为一个进行生物学和临床前研究的平台,该系统存在重要局限性。我们利用干扰素诱导型Mx1-Cre转基因在造血细胞中敲除条件性突变的Nf1等位基因。Nf1的体细胞失活会诱发一种具有100%外显率的骨髓增殖性疾病,该疾病与亚急性临床病程、髓样细胞的组织浸润、对粒细胞-巨噬细胞集落刺激因子的超敏反应、细胞过度增殖以及抗凋亡作用相关。这些Mx1-Cre、Nf1(flox/flox)小鼠建立了一个易于处理的实验模型,用于测试治疗方法以及识别在髓系白血病发生过程中与过度活跃的Ras协同作用的突变。

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