Fry Andrew M, Leaper Michelle J, Bayliss Richard
Department of Biochemistry; University of Leicester; Leicester, UK.
Organogenesis. 2014 Jan 1;10(1):62-8. doi: 10.4161/org.28910. Epub 2014 Apr 17.
The primary cilium is an antenna-like organelle that plays a vital role in organ generation and maintenance. It protrudes from the cell surface where it receives signals from the surrounding environment and relays them into the cell. These signals are then integrated to give the required outputs in terms of proliferation, differentiation, migration and polarization that ultimately lead to organ development and homeostasis. Defects in cilia function underlie a wide range of diverse but related human developmental or degenerative diseases. Collectively known as ciliopathies, these disorders present with varying severity and multiple organ involvement. The appreciation of the medical importance of the primary cilium has stimulated a huge effort into studies of the underlying cellular mechanisms. These in turn have revealed that ciliopathies result not only from defective assembly or organization of the primary cilium, but also from impaired ciliary signaling. This special edition of Organogenesis contains a set of review articles that highlight the role of the primary cilium in organ development and homeostasis, much of which has been learnt from studies of the associated human diseases. Here, we provide an introductory overview of our current understanding of the structure and function of the cilium, with a focus on the signaling pathways that are coordinated by primary cilia to ensure proper organ generation and maintenance.
初级纤毛是一种类似天线的细胞器，在器官生成和维持中起着至关重要的作用。它从细胞表面伸出，在那里接收来自周围环境的信号并将其传递到细胞内。这些信号随后被整合，以产生增殖、分化、迁移和极化方面所需的输出，最终导致器官发育和体内平衡。纤毛功能缺陷是多种不同但相关的人类发育或退行性疾病的基础。这些疾病统称为纤毛病，表现出不同的严重程度和多器官受累。对初级纤毛医学重要性的认识激发了对其潜在细胞机制的大量研究。这些研究反过来又揭示，纤毛病不仅源于初级纤毛组装或组织缺陷，还源于纤毛信号传导受损。本期《器官发生》特刊包含一组综述文章，突出了初级纤毛在器官发育和体内平衡中的作用，其中很多是从相关人类疾病研究中学到的。在此，我们对纤毛的结构和功能的当前理解进行简要概述，重点关注由初级纤毛协调以确保正确器官生成和维持的信号通路。
