Signaling in the primary cilium through the lens of the Hedgehog pathway.,WIREs Mechanisms of Disease

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Signaling in the primary cilium through the lens of the Hedgehog pathway.
WIREs Mechanisms of Disease ( IF 4.6 ) Pub Date : 2020-02-21 , DOI: 10.1002/wdev.377
Eduardo D Gigante ₁ , Tamara Caspary ₂

Affiliation

Cilia are microtubule‐based, cell‐surface projections whose machinery is evolutionarily conserved. In vertebrates, cilia are observed on almost every cell type and are either motile or immotile. Immotile sensory, or primary cilia, are responsive to extracellular ligands and signals. Cilia can be thought of as compartments, functionally distinct from the cell that provides an environment for signaling cascades. Hedgehog is a critical developmental signaling pathway which is functionally linked to primary cilia in vertebrates. The major components of the vertebrate Hedgehog signaling pathway dynamically localize to the ciliary compartment and ciliary membrane. Critically, G‐protein coupled receptor (GPCR) Smoothened, the obligate transducer of the pathway, is enriched and activated in the cilium. While Smoothened is the most intensely studied ciliary receptor, many GPCRs localize within cilia. Understanding the link between Smoothened and cilia defines common features, and distinctions, of GPCR signaling within the primary cilium.

中文翻译：

通过 Hedgehog 通路的晶状体在初级纤毛中发出信号。

纤毛是基于微管的细胞表面投射物，其机制在进化上是保守的。在脊椎动物中，几乎在每种细胞类型上都观察到纤毛，并且纤毛可以活动或不活动。不动的感觉或初级纤毛对细胞外配体和信号有反应。纤毛可以被认为是隔间，其功能不同于为信号级联提供环境的细胞。Hedgehog 是一种关键的发育信号通路，在功能上与脊椎动物的初级纤毛有关。脊椎动物 Hedgehog 信号通路的主要成分动态定位于睫状隔室和睫状膜。至关重要的是，G 蛋白偶联受体 (GPCR) Smoothened，通路的专性转导，在纤毛中富集和激活。虽然 Smoothened 是研究最深入的纤毛受体，但许多 GPCR 定位于纤毛内。了解 Smoothened 和纤毛之间的联系定义了初级纤毛内 GPCR 信号传导的共同特征和区别。

更新日期：2020-02-21

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