Cilia orientation and the fluid mechanics of development
Cilia orientation and the fluid mechanics of development
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DOI：
10.1016/j.ceb.2007.11.009
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发表时间：
2008-02-01
影响因子：
7.5
通讯作者：
Kintner, Christopher
中科院分区：
文献类型：
--
作者：
Marshall, Wallace F.;Kintner, Christopher
运动纤毛产生对发育和生理至关重要的大规模流体流动。纤毛运动缺陷会导致一系列疾病症状，包括支气管扩张、脑积水和内脏反位。然而，纤毛仅能活动并产生流动还不够，流动必须沿正确的方向驱动。产生正确定向的相干流需要纤毛相对于组织轴正确定向。遗传、分子和超微结构研究已经开始提出将纤毛定向与平面细胞极性（PCP）和其他远程位置线索联系起来的途径，并且还表明纤毛驱动的流动本身可以在产生纤毛的定向中发挥因果作用。在人类纤毛疾病患者中观察到纤毛方向错误，这表明方向缺陷可能构成一类新的纤毛病，在细胞生物学水平上具有独特的病因学。
Motile cilia produce large-scale fluid flows crucial for development and physiology. Defects in ciliary motility cause a range of disease symptoms including bronchiectasis, hydrocephalus, and situs inversus. However, it is not enough for cilia to be motile and generate a flow-the flow must be driven in the proper direction. Generation of properly directed coherent flow requires that the cilia are properly oriented relative to tissue axes. Genetic, molecular, and ultrastructural studies have begun to suggest pathways linking cilia orientation to planar cell polarity (PCP) and other long-range positional cues and also suggest that cilia-driven flow can itself play a causal role in orienting the cilia that create it. Errors in cilia orientation have been observed in human ciliary disease patients, suggesting that orientation defects may constitute a novel class of ciliopathies with a distinct etiology at the cell biological level.