Department of Neurology, Faculty of Medicine, University of Pécs, Rét utca 2, Pécs, 7623, Hungary.
J Mol Neurosci. 2018 Mar;64(3):397-409. doi: 10.1007/s12031-018-1036-z. Epub 2018 Feb 20.
Primary cilia are small, special cellular organelles that provide important sensory and signaling functions during the development of mammalian organs and coordination of postnatal cellular processes. Dysfunction of primary cilia are thought to be the main cause of ciliopathies, a group of genetic disorders characterized by overlapping developmental defects and prominent neurodevelopmental features. Although, disrupted cilia-linked signaling pathways have been implicated in the regulation of numerous neuronal functions, the precise role of primary cilia in the brain are still unknown. Importantly, studies of recent years have highlighted that different functions of primary cilia are reflected by their diverse morphology and unique signaling components localized in the ciliary membrane. In the present study, we conducted a comparative analysis of the expression pattern, distribution and length of adenylyl cyclase 3, somatostatin receptor 3, and ADP-ribosylation factor-like protein 13B expressing primary cilia in the mouse brain. We show that cilia of neurons and astrocytes display a well characterized distribution and ciliary marker arrangements. Moreover, quantitative comparison of their length, density and occurrence rate revealed that primary cilia exhibit region-specific alternations. In summary, our study provides a comprehensive overview of the cellular organization and morphological traits of primary cilia in regions of the physiological adult mouse brain.
初级纤毛是小型的特殊细胞细胞器,在哺乳动物器官的发育和出生后细胞过程的协调中提供重要的感觉和信号功能。初级纤毛功能障碍被认为是纤毛病的主要原因,纤毛病是一组遗传疾病,其特征是重叠的发育缺陷和突出的神经发育特征。尽管与纤毛相关的信号通路的破坏已被牵连到许多神经元功能的调节中,但初级纤毛在大脑中的精确作用仍不清楚。重要的是,近年来的研究强调,初级纤毛的不同功能反映在其不同的形态和定位于纤毛膜中的独特信号成分上。在本研究中,我们对表达腺苷酸环化酶 3、生长抑素受体 3 和 ADP-核糖基化因子样蛋白 13B 的初级纤毛在小鼠大脑中的表达模式、分布和长度进行了比较分析。我们表明,神经元和星形胶质细胞的纤毛显示出特征性的分布和纤毛标记排列。此外,对其长度、密度和发生率的定量比较表明,初级纤毛表现出区域特异性的改变。总之,我们的研究提供了生理成年小鼠大脑区域中初级纤毛的细胞组织和形态特征的全面概述。