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首页> 外文期刊>PLoS Genetics >Rsph4a is essential for the triplet radial spoke head assembly of the mouse motile cilia
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Rsph4a is essential for the triplet radial spoke head assembly of the mouse motile cilia

机译：RSPH4A对于鼠标Motile Cilia的三联径向辐条头组件至关重要

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Motile cilia/flagella are essential for swimming and generating extracellular fluid flow in eukaryotes. Motile cilia harbor a 9+2 arrangement consisting of nine doublet microtubules with dynein arms at the periphery and a pair of singlet microtubules at the center (central pair). In the central system, the radial spoke has a T-shaped architecture and regulates the motility and motion pattern of cilia. Recent cryoelectron tomography data reveal three types of radial spokes (RS1, RS2, and RS3) in the 96 nm axoneme repeat unit; however, the molecular composition of the third radial spoke, RS3 is unknown. In human pathology, it is well known mutation of the radial spoke head-related genes causes primary ciliary dyskinesia (PCD) including respiratory defect and infertility. Here, we describe the role of the primary ciliary dyskinesia protein Rsph4a in the mouse motile cilia. Cryoelectron tomography reveals that the mouse trachea cilia harbor three types of radial spoke as with the other vertebrates and that all triplet spoke heads are lacking in the trachea cilia of Rsph4a-deficient mice. Furthermore, observation of ciliary movement and immunofluorescence analysis indicates that Rsph4a contributes to the generation of the planar beating of motile cilia by building the distal architecture of radial spokes in the trachea, the ependymal tissues, and the oviduct. Although detailed mechanism of RSs assembly remains unknown, our results suggest Rsph4a is a generic component of radial spoke heads, and could explain the severe phenotype of human PCD patients with RSPH4A mutation.
机译：Motile Cilia /鞭毛对于游泳和产生真核生物的细胞外液流是必不可少的。 Motile Cilia Harbour A 9 + 2布置，由九个双链微管组成，在周边，在中心（中央对）处的一对单向微管。在中央系统中，径向辐条具有T形架构，并调节纤毛的运动和运动模式。最近的低温电压术数据显示在96nm Axoneme重复单元中的三种类型的径向辐条（RS1，RS2和RS3）;然而，第三径向辐条的分子组成，RS3是未知的。在人体病理学中，径向辐条头颈相关基因的众所周知是引起初级睫状体缺陷（PCD），包括呼吸缺陷和不孕症。在这里，我们描述了原发性睫状体蛋白质蛋白RSPH4a在小鼠运动纤毛中的作用。冷冻电子断层扫描揭示了小鼠气管Cilia港口三种类型的径向辐条与其他脊椎动物一样，并且所有三重态辐条头都缺乏RSPh4a缺陷小鼠的气管纤毛。此外，观察睫状体运动和免疫荧光分析表明，RSPH4A通过在气管中的径向辐条和输卵管和输卵管中构建径向辐条的远端架构来产生运动纤毛的平面搏动。尽管RSS组件的详细机制仍然是未知的，但我们的结果表明RSPH4A是径向辐条头的通用组件，并且可以解释患有RSPH4A突变的人PCD患者的严重表型。

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