Department of Biomedicine and Prevention, University of Rome Tor Vergata, Rome, Italy.
Fondazione Santa Lucia, IRCCS, Rome, Italy.
Cell Mol Life Sci. 2021 Oct;78(19-20):6431-6451. doi: 10.1007/s00018-021-03911-w. Epub 2021 Aug 18.
In the past two decades, mounting evidence has modified the classical view of the cerebellum as a brain region specifically involved in the modulation of motor functions. Indeed, clinical studies and engineered mouse models have highlighted cerebellar circuits implicated in cognitive functions and behavior. Furthermore, it is now clear that insults occurring in specific time windows of cerebellar development can affect cognitive performance later in life and are associated with neurological syndromes, such as Autism Spectrum Disorder. Despite its almost homogenous cytoarchitecture, how cerebellar circuits form and function is not completely elucidated yet. Notably, the apparently simple neuronal organization of the cerebellum, in which Purkinje cells represent the only output, hides an elevated functional diversity even within the same neuronal population. Such complexity is the result of the integration of intrinsic morphogenetic programs and extracellular cues from the surrounding environment, which impact on the regulation of the transcriptome of cerebellar neurons. In this review, we briefly summarize key features of the development and structure of the cerebellum before focusing on the pathways involved in the acquisition of the cerebellar neuron identity. We focus on gene expression and mRNA processing programs, including mRNA methylation, trafficking and splicing, that are set in motion during cerebellar development and participate to its physiology. These programs are likely to add new layers of complexity and versatility that are fundamental for the adaptability of cerebellar neurons.
在过去的二十年中,越来越多的证据改变了经典的小脑观点,即小脑是专门参与调节运动功能的脑区。事实上,临床研究和工程化小鼠模型强调了与认知功能和行为相关的小脑回路。此外,现在很清楚,小脑发育特定时间窗内发生的损伤会影响以后的认知表现,并与神经综合征相关,如自闭症谱系障碍。尽管小脑具有几乎同质的细胞结构,但小脑回路如何形成和发挥作用尚不完全清楚。值得注意的是,小脑表面上简单的神经元组织,其中浦肯野细胞是唯一的输出,隐藏着即使在同一神经元群体中也存在的高度功能多样性。这种复杂性是内在形态发生程序与来自周围环境的细胞外线索整合的结果,这影响了小脑神经元转录组的调节。在这篇综述中,我们简要总结了小脑发育和结构的关键特征,然后重点介绍了获得小脑神经元身份的相关途径。我们关注的是基因表达和 mRNA 处理程序,包括 mRNA 甲基化、运输和剪接,这些程序在小脑发育过程中启动,并参与小脑的生理功能。这些程序可能会增加小脑神经元适应性的新的复杂性和多功能性。