Motile cilia of multiciliated epithelial cells undergo synchronized beating to produce fluid flow along the luminal surface of various organs. Each motile cilium consists of an axoneme and a basal body, which are linked by a ‘transition zone’. The axoneme exhibits a characteristic 9+2 microtubule arrangement important for ciliary motion, but how this microtubule system is generated is not yet fully understood. Here we show that CAMSAP3, a protein that can stabilize the minus end of a microtubule, concentrates at multiple sites of the cilium–basal body complex, including the upper region of the transition zone or the axonemal basal plate where the central pair of microtubules (CP) initiates. CAMSAP3 dysfunction resulted in loss of the CP and partial distortion of the basal plate, as well as the failure of multicilia to undergo synchronized beating. These findings suggest that CAMSAP3 plays pivotal roles in the formation or stabilization of the CP by localizing at the basal region of the axoneme, and thereby supports the coordinated motion of multicilia in airway epithelial cells.

多纤毛上皮细胞的活动纤毛经历同步跳动以产生沿各种器官的腔表面流动的流体。每个活动纤毛由一个轴丝和一个基体组成，它们由一个“过渡区”连接。轴丝表现出对纤毛运动很重要的特征性 9+2 微管排列，但尚未完全了解该微管系统是如何产生的。在这里，我们表明 CAMSAP3 是一种可以稳定微管负端的蛋白质，它集中在纤毛 - 基底复合体的多个部位，包括过渡区的上部区域或轴突基板，其中中央对微管。 CP) 发起。CAMSAP3 功能障碍导致 CP 丧失和基底板部分变形，以及多纤毛不能进行同步跳动。