The primary cilium: guardian of organ development and homeostasis.
The primary cilium: guardian of organ development and homeostasis.
复制标题
主要纤毛：器官开发和体内平衡的守护者。
DOI：
10.4161/org.28910
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发表时间：
2014-01-01
期刊：
影响因子：
2.3
通讯作者：
Bayliss R
中科院分区：
文献类型：
--
作者：
Fry AM;Leaper MJ;Bayliss R
初级纤毛是一种类似触角的细胞器，在器官的生成和维持中起着至关重要的作用。它从细胞表面伸出，在那里它接收来自周围环境的信号，并将它们传递到细胞内。然后，这些信号被整合起来，在增殖、分化、迁移和极化方面提供所需的输出，最终导致器官发育和动态平衡。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。统称为纤毛疾病，这些疾病表现出不同的严重程度和多器官受累。对初级纤毛的医学重要性的认识刺激了人们对潜在细胞机制的巨大研究。这些反过来揭示了纤毛病变不仅是由于初级纤毛的组装或组织缺陷所致，也是由于纤毛信号受损所致。这一特别版的器官发生包含一组综述文章，强调初级纤毛在器官发育和动态平衡中的作用，其中大部分是从相关人类疾病的研究中学到的。在这里，我们提供一个介绍性的概述我们目前对纤毛的结构和功能的理解，重点放在初级纤毛协调的信号通路上，以确保适当的器官生成和维持。
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.
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影响因子：
--
作者：
Carroll TJ;Yu J
通讯作者：
Yu J
影响因子：
64.5
作者：
Chaki M;Airik R;Ghosh AK;Giles RH;Chen R;Slaats GG;Wang H;Hurd TW;Zhou W;Cluckey A;Gee HY;Ramaswami G;Hong CJ;Hamilton BA;Cervenka I;Ganji RS;Bryja V;Arts HH;van Reeuwijk J;Oud MM;Letteboer SJ;Roepman R;Husson H;Ibraghimov-Beskrovnaya O;Yasunaga T;Walz G;Eley L;Sayer JA;Schermer B;Liebau MC;Benzing T;Le Corre S;Drummond I;Janssen S;Allen SJ;Natarajan S;O'Toole JF;Attanasio M;Saunier S;Antignac C;Koenekoop RK;Ren H;Lopez I;Nayir A;Stoetzel C;Dollfus H;Massoudi R;Gleeson JG;Andreoli SP;Doherty DG;Lindstrad A;Golzio C;Katsanis N;Pape L;Abboud EB;Al-Rajhi AA;Lewis RA;Omran H;Lee EY;Wang S;Sekiguchi JM;Saunders R;Johnson CA;Garner E;Vanselow K;Andersen JS;Shlomai J;Nurnberg G;Nurnberg P;Levy S;Smogorzewska A;Otto EA;Hildebrandt F
通讯作者：
Hildebrandt F
影响因子：
64.8
作者：
Barr, MM;Sternberg, PW
通讯作者：
Sternberg, PW
影响因子：
30.8
作者：
Gerdes, Jantje M.;Liu, Yangfan;Katsanis, Nicholas
通讯作者：
Katsanis, Nicholas
影响因子：
19
作者：
Czarnecki, Peter G.;Shah, Jagesh V.
通讯作者：
Shah, Jagesh V.