Pulikkan J A, Tenen D G, Behre G
Department of Molecular, Cell and Cancer Biology, University of Massachusetts Medical School, Worcester, MA, USA.
Cancer Science Institute, National University of Singapore, Singapore, Singapore.
Leukemia. 2017 Nov;31(11):2279-2285. doi: 10.1038/leu.2017.229. Epub 2017 Jul 19.
Myeloid master regulator CCAAT enhancer-binding protein alpha (C/EBPα) is deregulated by multiple mechanisms in leukemia. Inhibition of C/EBPα function plays pivotal roles in leukemogenesis. While much is known about how C/EBPα orchestrates granulopoiesis, our understanding of molecular transformation events, the role(s) of cooperating mutations and clonal evolution during C/EBPα deregulation in leukemia remains elusive. In this review, we will summarize the latest research addressing these topics with special emphasis on CEBPA mutations. We conclude by describing emerging therapeutic strategies to restore C/EBPα function.
髓系主调控因子CCAAT增强子结合蛋白α（C/EBPα）在白血病中通过多种机制发生失调。抑制C/EBPα功能在白血病发生过程中起关键作用。虽然我们对C/EBPα如何调控粒细胞生成了解很多，但对于白血病中C/EBPα失调期间的分子转化事件、协同突变的作用以及克隆进化，我们仍知之甚少。在这篇综述中，我们将总结针对这些主题的最新研究，特别强调CEBPA突变。我们通过描述恢复C/EBPα功能的新兴治疗策略来结束本文。
