Schüler Andrea, Schwieger Maike, Engelmann Afra, Weber Kristoffer, Horn Stefan, Müller Ursula, Arnold Michael A, Olson Eric N, Stocking Carol
Heinrich-Pette-Institute, Hamburg, Germany.
Blood. 2008 May 1;111(9):4532-41. doi: 10.1182/blood-2007-10-116343. Epub 2008 Mar 7.
Mef2c is a MADS (MCM1-agamous-deficient serum response factor) transcription factor best known for its role in muscle and cardiovascular development. A causal role of up-regulated MEF2C expression in myelomonocytic acute myeloid leukemia (AML) has recently been demonstrated. Due to the pronounced monocytic component observed in Mef2c-induced AML, this study was designed to assess the importance of Mef2c in normal myeloid differentiation. Analysis of bone marrow (BM) cells manipulated to constitutively express Mef2c demonstrated increased monopoiesis at the expense of granulopoiesis, whereas BM isolated from Mef2c(Delta/-) mice showed reduced levels of monocytic differentiation in response to cytokines. Mechanistic studies showed that loss of Mef2c expression correlated with reduced levels of transcripts encoding c-Jun, but not PU.1, C/EBPalpha, or JunB transcription factors. Inhibiting Jun expression by short-interfering RNA impaired Mef2c-mediated inhibition of granulocyte development. Moreover, retroviral expression of c-Jun in BM cells promoted monocytic differentiation. The ability of Mef2c to modulate cell-fate decisions between monocyte and granulocyte differentiation, coupled with its functional sensitivity to extracellular stimuli, demonstrate an important role in immunity--and, consistent with findings of other myeloid transcription factors, a target of oncogenic lesions in AML.
Mef2c是一种MADS(MCM1-无配子-血清反应因子缺陷)转录因子,因其在肌肉和心血管发育中的作用而广为人知。最近已证明上调的MEF2C表达在髓单核细胞性急性髓系白血病(AML)中具有因果作用。由于在Mef2c诱导的AML中观察到明显的单核细胞成分,本研究旨在评估Mef2c在正常髓系分化中的重要性。对经操作组成性表达Mef2c的骨髓(BM)细胞的分析表明,以粒细胞生成减少为代价,单核细胞生成增加,而从Mef2c(Delta/-)小鼠分离的BM对细胞因子的反应显示单核细胞分化水平降低。机制研究表明,Mef2c表达的缺失与编码c-Jun的转录本水平降低相关,但与PU.1、C/EBPalpha或JunB转录因子无关。通过短干扰RNA抑制Jun表达会损害Mef2c介导的粒细胞发育抑制。此外,BM细胞中c-Jun的逆转录病毒表达促进了单核细胞分化。Mef2c调节单核细胞和粒细胞分化之间细胞命运决定的能力,及其对细胞外刺激的功能敏感性,表明其在免疫中起重要作用——并且与其他髓系转录因子的研究结果一致,是AML致癌病变的一个靶点。