Trafficking in and to the primary cilium.
Trafficking in and to the primary cilium.
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DOI：
10.1186/2046-2530-1-4
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发表时间：
2012-04-25
期刊：
影响因子：
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通讯作者：
Ferland RJ
中科院分区：
文献类型：
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作者：
Hsiao YC;Tuz K;Ferland RJ
极化囊泡运输是由小的GTPase蛋白介导的，如Rabs和Arls/Arfs。这些蛋白在维持正常细胞功能方面发挥重要作用，部分是通过调节细胞内运输。此外，这些蛋白质家族最近被认为与初级纤毛的形成和功能有关。初级纤毛几乎存在于脊椎动物的每一种细胞中，它是一种从细胞表面突出的细胞器，起着信号传递中心的作用。有趣的是，它最近被与多种人类疾病联系在一起，统称为纤毛病。初级纤毛在其膜上具有异常高密度的受体，这些受体对于感知和传导细胞外刺激非常重要。此外，初级纤毛作为一个独立于细胞质的细胞室，为信号分子启动下游事件提供独特的空间和时间调节。因此，功能正常的初级纤毛对正常的信号转导至关重要。Rabs和Arls/Arfs在早期纤毛形成中发挥关键作用，但也需要通过与初级纤毛的专门运输系统鞭毛内运输（IFT）协调来维持纤毛功能。纤毛中的IFT对于蛋白质进出这种高度调节的细胞器的适当运动至关重要。在这篇综述文章中，我们探讨了极化囊泡运输在纤毛形成和功能中的作用，并讨论了这些过程中的缺陷如何导致纤毛病中观察到的异常。
Polarized vesicle trafficking is mediated by small GTPase proteins, such as Rabs and Arls/Arfs. These proteins have essential roles in maintaining normal cellular function, in part, through regulating intracellular trafficking. Moreover, these families of proteins have recently been implicated in the formation and function of the primary cilium. The primary cilium, which is found on almost every cell type in vertebrates, is an organelle that protrudes from the surface of the cell and functions as a signaling center. Interestingly, it has recently been linked to a variety of human diseases, collectively referred to as ciliopathies. The primary cilium has an exceptionally high density of receptors on its membrane that are important for sensing and transducing extracellular stimuli. Moreover, the primary cilium serves as a separate cellular compartment from the cytosol, providing for unique spatial and temporal regulation of signaling molecules to initiate downstream events. Thus, functional primary cilia are essential for normal signal transduction. Rabs and Arls/Arfs play critical roles in early cilia formation but are also needed for maintenance of ciliary function through their coordination with intraflagellar transport (IFT), a specialized trafficking system in primary cilia. IFT in cilia is pivotal for the proper movement of proteins into and out of this highly regulated organelle. In this review article, we explore the involvement of polarized vesicular trafficking in cilia formation and function, and discuss how defects in these processes could subsequently lead to the abnormalities observed in ciliopathies.