The establishment of rotational polarity in the airway and ependymal cilia: analysis with a novel cilium motility mutant mouse
The establishment of rotational polarity in the airway and ependymal cilia: analysis with a novel cilium motility mutant mouse
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DOI：
10.1152/ajplung.00425.2012
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发表时间：
2013-06-01
影响因子：
4.9
通讯作者：
Takeda, Hiroyuki
中科院分区：
文献类型：
--
作者：
Matsuo, Moe;Shimada, Atsuko;Takeda, Hiroyuki
呼吸道被复纤毛覆盖，复纤毛以异时的方式向口腔搏动，以清除碎屑和感染性颗粒。协调的单向搏动是气道中多纤毛的基本特征，以保证适当的粘膜纤毛清除。纤毛运动缺陷导致原发性纤毛运动障碍（PCD），主要症状包括支气管炎和其他慢性呼吸道疾病。最近的工作表明，纤毛运动和平面极性的过程中所需的纤毛排列，产生协调跳动。然而，纤毛运动在哺乳动物中参与这一过程的程度尚未完全阐明。在这里，为了解决纤毛运动在协调纤毛排列过程中的作用，我们分析了Kintoun小鼠突变体（Ktu（-/-））。Ktu（-/-）表现出典型的PCD表型，气管和另一纤毛组织和室管膜中纤毛运动完全丧失。免疫组化使用轴丝动力蛋白的抗体证实了多个轴丝动力蛋白突变体纤毛组件的损失。纤毛方向的基础上基足方向的观察显示，纤毛运动是不需要在气道纤毛的对齐，而在大脑室管膜细胞中观察到一个强烈的要求。因此，我们得出结论，参与睫状体运动在建立协调的纤毛排列各不相同的组织。
The airway is covered by multicilia that beat in a metachronous manner toward the mouth to eliminate debris and infectious particles. Coordinated one-directional beating is an essential feature of multicilia in the airway to guarantee proper mucociliary clearance. Defects in ciliary motility lead to primary ciliary dyskinesia (PCD), with major symptoms including bronchitis and other chronic respiratory diseases. Recent work suggested that ciliary motility and planar polarity are required in the process of ciliary alignment that produces coordinated beating. However, the extent to which cilia motility is involved in this process in mammals has not yet been fully clarified. Here, to address the role of ciliary motility in the process of coordinated ciliary alignment, we analyzed Kintoun mice mutants (Ktu(-/-)). Ktu(-/-) exhibited typical phenotypes of PCD with complete loss of ciliary motility in trachea and another ciliated tissue, the brain ependyma. Immunohistochemistry using antibodies against axonemal dynein confirmed the loss of multiple axonemal dynein components in mutant cilia. Observation of cilia orientation based on basal foot directions revealed that ciliary motility was not required in the alignment of airway cilia, whereas a strong requirement was observed in brain ependymal cells. Thus we conclude that the involvement of ciliary motility in the establishment of coordinated ciliary alignment varies among tissues.