Department of Pediatrics 1, Martin Luther University Halle-Wittenberg, 06120 Halle, Germany.
Institute of Molecular Medicine, Martin Luther University Halle-Wittenberg, 06120 Halle, Germany.
Int J Mol Sci. 2020 May 12;21(10):3409. doi: 10.3390/ijms21103409.
Acute leukemias are genetic diseases caused by translocations or mutations, which dysregulate hematopoiesis towards malignant transformation. However, the molecular mode of action is highly versatile and ranges from direct transcriptional to post-transcriptional control, which includes RNA-binding proteins (RBPs) as crucial regulators of cell fate. RBPs coordinate RNA dynamics, including subcellular localization, translational efficiency and metabolism, by binding to their target messenger RNAs (mRNAs), thereby controlling the expression of the encoded proteins. In view of the growing interest in these regulators, this review summarizes recent research regarding the most influential RBPs relevant in acute leukemias in particular. The reported RBPs, either dysregulated or as components of fusion proteins, are described with respect to their functional domains, the pathways they affect, and clinical aspects associated with their dysregulation or altered functions.
急性白血病是由易位或突变引起的遗传疾病,这些易位或突变导致造血向恶性转化失调。然而,分子作用模式非常多样化,范围从直接转录到转录后控制,其中包括 RNA 结合蛋白 (RBPs) 作为细胞命运的关键调节剂。RBPs 通过与靶信使 RNA(mRNA)结合来协调 RNA 的动态变化,包括亚细胞定位、翻译效率和代谢,从而控制编码蛋白的表达。鉴于人们对这些调节剂的兴趣日益增加,本综述总结了最近关于急性白血病中特别相关的最有影响力的 RBPs 的研究。报告的 RBPs,无论是失调还是作为融合蛋白的组成部分,都根据其功能域、它们影响的途径以及与它们失调或功能改变相关的临床方面进行了描述。