Christensen Søren T, Morthorst Stine K, Mogensen Johanne B, Pedersen Lotte B
Department of Biology, University of Copenhagen, DK-2100 Copenhagen OE, Denmark.
Cold Spring Harb Perspect Biol. 2017 Jun 1;9(6):a028167. doi: 10.1101/cshperspect.a028167.
Since the beginning of the millennium, research in primary cilia has revolutionized our way of understanding how cells integrate and organize diverse signaling pathways during vertebrate development and in tissue homeostasis. Primary cilia are unique sensory organelles that detect changes in their extracellular environment and integrate and transmit signaling information to the cell to regulate various cellular, developmental, and physiological processes. Many different signaling pathways have now been shown to rely on primary cilia to function properly, and mutations that lead to ciliary dysfunction are at the root of a pleiotropic group of diseases and syndromic disorders called ciliopathies. In this review, we present an overview of primary cilia-mediated regulation of receptor tyrosine kinase (RTK) and transforming growth factor β (TGF-β) signaling. Further, we discuss how defects in the coordination of these pathways may be linked to ciliopathies.
自千禧年伊始，对初级纤毛的研究彻底改变了我们理解细胞在脊椎动物发育和组织稳态过程中如何整合和组织多种信号通路的方式。初级纤毛是独特的感觉细胞器，可检测细胞外环境的变化，并整合信号信息并将其传递给细胞，以调节各种细胞、发育和生理过程。现已表明，许多不同的信号通路都依赖初级纤毛来正常发挥功能，导致纤毛功能障碍的突变是一组多效性疾病和综合征性疾病（称为纤毛病）的根源。在本综述中，我们概述了初级纤毛介导的受体酪氨酸激酶（RTK）和转化生长因子β（TGF-β）信号传导的调节。此外，我们讨论了这些信号通路协调缺陷如何可能与纤毛病相关联。
