Smithson Laura J, Anastasaki Corina, Chen Ran, Toonen Joseph A, Williams Sidney B, Gutmann David H
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, United States.
Department of Neurology, Washington University School of Medicine, St. Louis, MO 63110, United States.
Semin Cell Dev Biol. 2016 Oct;58:118-26. doi: 10.1016/j.semcdb.2016.06.002. Epub 2016 Jun 3.
The formation and maintenance of an organism are highly dependent on the orderly control of cell growth, differentiation, death, and migration. These processes are tightly regulated by signaling cascades in which a limited number of molecules dictate these cellular events. While these signaling pathways are highly conserved across species and cell types, the functional outcomes that result from their engagement are specified by the context in which they are activated. Using the Neurofibromatosis type 1 (NF1) cancer predisposition syndrome as an illustrative platform, we discuss how NF1/RAS signaling can create functional diversity at multiple levels (molecular, cellular, tissue, and genetic/genomic). As such, the ability of related molecules (e.g., K-RAS, H-RAS) to activate distinct effectors, as well as cell type- and tissue-specific differences in molecular composition and effector engagement, generate numerous unique functional effects. These variations, coupled with a multitude of extracellular cues and genomic/genetic changes that each modify the innate signaling properties of the cell, enable precise control of cellular physiology in both health and disease. Understanding these contextual influences is important when trying to dissect the underlying pathogenic mechanisms of cancer relevant to molecularly-targeted therapeutics.
生物体的形成和维持高度依赖于对细胞生长、分化、死亡和迁移的有序控制。这些过程由信号级联严格调控,其中有限数量的分子决定这些细胞事件。虽然这些信号通路在物种和细胞类型之间高度保守,但它们激活所产生的功能结果由其被激活的背景所决定。以1型神经纤维瘤病(NF1)癌症易感综合征作为一个说明性平台,我们讨论NF1/RAS信号如何在多个层面(分子、细胞、组织以及遗传/基因组)产生功能多样性。因此,相关分子(如K-RAS、H-RAS)激活不同效应器的能力,以及分子组成和效应器参与方面的细胞类型和组织特异性差异,产生了众多独特的功能效应。这些变异,再加上众多改变细胞固有信号特性的细胞外信号和基因组/遗传变化,使得在健康和疾病状态下都能精确控制细胞生理。在试图剖析与分子靶向治疗相关的癌症潜在致病机制时,了解这些背景影响非常重要。