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首页> 外文期刊>Cell cycle >Chibby functions to preserve normal ciliary morphology through the regulation of intraflagellar transport in airway ciliated cells
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Chibby functions to preserve normal ciliary morphology through the regulation of intraflagellar transport in airway ciliated cells

机译：通过调节气道纤毛细胞鞭毛内运输，Chibby功能可保持正常的睫状形态

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摘要

Airway cilia provide the coordinated motive force for mucociliary transport, which prevents the accumulation of mucus, debris, pollutants, and bacteria in our respiratory tracts. As airway cilia are constantly exposed to the environment and, hence, are an integral component of the pathogenesis of several congenital and chronic pulmonary disorders, it is necessary to understand the molecular mechanisms that control ciliated cell differentiation and ciliogenesis. We have previously reported that loss of the basal body protein Chibby (Cby) results in chronic upper airway infection in mice due to a significant reduction in the number of airway cilia. In the present work, we demonstrate that Cby is required for normal ciliary structure and proper distribution of proteins involved in the bidirectional intraflagellar transport (IFT) system, which consists of 2 distinct sub-complexes, IFT-A and IFT-B, and is essential for ciliary biogenesis and maintenance. In fully differentiated ciliated cells, abnormal paddle-like cilia with dilated ciliary tips are observed in Cby-/- airways and primary cultures of mouse tracheal epithelial cells (MTECs). In addition, IFT88, an IFT-B sub-complex protein, robustly accumulates within the dilated tips of both multicilia in Cby-/- MTECs and primary cilia in Cby-/- mouse embryonic fibroblasts (MEFs). Furthermore, we show that only IFT-B components, including IFT20 and IFT57, but not IFT-A and Bardet-Biedl syndrome (BBS) proteins, amass with IFT88 in these distended tips in Cby-/- ciliated cells. Taken together, our findings suggest that Cby plays a role in the proper distribution of IFT particles to preserve normal ciliary morphology in airway ciliated cells.
机译：气道纤毛为粘膜纤毛运输提供了协调的动力，可防止粘液，碎屑，污染物和细菌在呼吸道中积聚。由于气道纤毛一直暴露在环境中，因此是几种先天性和慢性肺部疾病发病机制的组成部分，因此有必要了解控制纤毛细胞分化和纤毛发生的分子机制。我们先前曾报道，由于气道纤毛数量的明显减少，失去了基体蛋白Chibby（Cby）会导致小鼠慢性上呼吸道感染。在目前的工作中，我们证明Cby是正常睫状结构和参与双向鞭毛内运输（IFT）系统的蛋白质的正确分布所必需的，该系统由2个不同的亚复合物IFT-A和IFT-B组成，并且对于睫毛的生物发生和维持至关重要。在完全分化的纤毛细胞中，在Cby-/-气道和小鼠气管上皮细胞（MTEC）的原代培养物中观察到异常的桨状纤毛和纤毛状纤毛。此外，IFT88，一种IFT-B亚复合蛋白，在Cby-/-MTECs的多纤毛和Cby-/-小鼠胚胎成纤维细胞（MEFs）的原发性纤毛的扩张末端牢固地积累。此外，我们显示只有IFT-B组件（包括IFT20和IFT57），而不是IFT-A和Bardet-Biedl综合征（BBS）蛋白，在Cby-/-纤毛细胞的这些扩展尖端中聚集了IFT88。综上所述，我们的研究结果表明Cby在IFT颗粒的正确分布中发挥作用，以保持气道纤毛细胞中正常的纤毛形态。

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