Role of intraflagellar transport and primary cilia in skeletal development
Role of intraflagellar transport and primary cilia in skeletal development
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DOI：
10.1002/ar.20634
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发表时间：
2008-09-01
影响因子：
2
通讯作者：
Serra, Rosa
中科院分区：
文献类型：
--
作者：
Serra, Rosa
初级纤毛是从几乎所有脊椎动物细胞表面延伸的基于微管的非运动性附属物。鞭毛内转运（IFT）过程负责初级纤毛的结构和功能的建立和维持。Kif 3a是驱动蛋白-II运动复合体的一个组成部分，其破坏使顺行IFT失效，并导致纤毛形成和维持失败。同样地，IFT颗粒的核心组分IFT 88/Tg 737/Polaris的缺失导致纤毛的损失。虽然近40年前在软骨细胞上描述了初级纤毛，但直到最近才通过使用含有制造和维持纤毛所需基因突变或缺失的小鼠模型揭示了IFT和纤毛在骨骼发育中的功能意义。总之，结果表明，初级纤毛/IFT参与协调骨骼内的多种信号通路。
Primary cilia are nonmotile microtubule-based appendages extending from the surface of almost all vertebrate cells. The process of intraflagellar transport (IFT) is responsible for building and maintaining the structure and function of primary cilia. Disruption of Kif3a, a component of the Kinesin-II motor complex, disables anterograde IFT and leads to failure in the formation and maintenance of cilia. Likewise, the absence of IFT88 / Tg737 /Polaris, a core component of the IFT particle, results in the loss of cilia. Although primary cilia were described on chondrocytes almost 40 years ago, only recently has the functional significance of IFT and cilia in skeletal development been uncovered through the use of mouse models containing mutations or deletions in genes required to make and maintain cilia. Together, the results indicate that primary cilia/IFT are involved in coordinating multiple signaling pathways within the skeleton.