Department of Cell Biology, Erasmus MC, Rotterdam, The Netherlands.
Int Rev Cell Mol Biol. 2013;303:101-38. doi: 10.1016/B978-0-12-407697-6.00003-9.
Primary cilia are highly conserved sensory organelles that extend from the surface of almost all vertebrate cells. The importance of cilia is evident from their involvement in many diseases, called ciliopathies. Primary cilia contain a microtubular axoneme that is used as a railway for transport of both structural components and signaling proteins. This transport machinery is called intraflagellar transport (IFT). Cilia are dynamic organelles whose presence on the cell surface, morphology, length and function are highly regulated. It is clear that the IFT machinery plays an important role in this regulation. However, it is not clear how, for example environmental cues or cell fate decisions are relayed to modulate IFT and cilium morphology or function. This chapter presents an overview of molecules that have been shown to regulate cilium length and IFT. Several examples where signaling modulates IFT and cilium function are used to discuss the importance of these systems for the cell and for understanding of the etiology of ciliopathies.
原发性纤毛是高度保守的感觉细胞器，从几乎所有脊椎动物细胞的表面伸出。纤毛在许多疾病（称为纤毛病）中的作用表明了它们的重要性。原发性纤毛包含一个微管轴丝，用作运输结构成分和信号蛋白的铁路。这种运输机制称为鞭毛内运输（IFT）。纤毛是动态细胞器，其在细胞表面的存在、形态、长度和功能受到高度调节。很明显，IFT 机制在这种调节中起着重要作用。然而，尚不清楚如何将环境线索或细胞命运决定传递到调节 IFT 和纤毛形态或功能。本章概述了已显示可调节纤毛长度和 IFT 的分子。使用几个信号调节 IFT 和纤毛功能的例子来讨论这些系统对细胞和理解纤毛病病因的重要性。
