Exploring the Activities of RBPMS Proteins in Myocardial Biology
Exploring the Activities of RBPMS Proteins in Myocardial Biology
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DOI：
10.1007/s00246-019-02180-6
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发表时间：
2019-10-01
影响因子：
1.6
通讯作者：
Burns, C. Geoffrey
中科院分区：
文献类型：
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作者：
Akerberg, Alexander A.;Burns, Caroline E.;Burns, C. Geoffrey
许多RNA结合蛋白（RBP）在心脏中表达，通过遗传关联、动物模型和机制研究，几种RBP的突变与心血管疾病有关。然而，更多的心脏RBP的功能，以及它们与疾病状态的相关性，仍有待阐明。最近，我们已经启动了研究，以表征RBPs RBPMS和RBPMS 2在调节斑马鱼和高等脊椎动物物种的心肌生物学中的功能。这些研究开始于我们使用无偏见的基因发现方法了解到斑马鱼的rbpms 2a和rbpms 2b是胚胎心肌的可靠标记。这一观察结果与已发表的数据一致，表明编码的蛋白质可能在调节心肌细胞分化、增殖、存活和/或收缩性的一个或多个方面中执行关键功能。这一观点得到了最近报告的支持，这些报告表明，Rbpms 2功能受损的斑马鱼胚胎表现出心脏窘迫的明显迹象。有趣的是，一项20年前的研究确定，来自青蛙、小鸡和小鼠的心肌组织也表达高水平的Rbpms和/或Rbpms 2，这表明功能的进化保守性。在这篇综述中，我们将提供RBPMS和RBPMS 2基因是如何被发现的历史记录，试图澄清一些潜在的混淆命名，并总结已发表的观察结果，为我们正在进行的研究提供信息。
Numerous RNA-binding proteins (RBPs) are expressed in the heart, and mutations in several RBPs have been implicated in cardiovascular disease through genetic associations, animal modeling, and mechanistic studies. However, the functions of many more cardiac RBPs, and their relevance to disease states, remain to be elucidated. Recently, we have initiated studies to characterize the functions of the RBPs RBPMS and RBPMS2 in regulating myocardial biology in zebrafish and higher vertebrate species. These studies began when we learned, using an unbiased gene discovery approach, that rbpms2a and rbpms2b in zebrafish are robust markers of embryonic myocardium. This observation, which is consistent with published data, suggests that the encoded proteins are likely to be performing critical functions in regulating one or more aspects of cardiomyocyte differentiation, proliferation, survival, and/or contractility. This notion is supported by recent reports demonstrating that zebrafish embryos with disrupted Rbpms2 function exhibit gross signs of cardiac distress. Interestingly, a 20-year-old study determined that myocardial tissue from the frog, chick, and mouse also express high levels of Rbpms and/or Rbpms2, which is suggestive of evolutionary conservation of function. In this review, we will provide a historical account of how RBPMS and RBPMS2 genes were discovered, attempt to clarify some potentially confusing nomenclature, and summarize published observations that inform our ongoing studies.