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Role for intraflagellar transport in building a functional transition zone

机译：在构建功能过渡区时，在构建功能过渡区的内际交通的作用

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Synopsis Genetic disorders caused by cilia dysfunction, termed ciliopathies, frequently involve the intraflagellar transport (IFT) system. Mutations in IFT subunits-including IFT-dynein motor DYNC2H1-impair ciliary structures and Hedgehog signalling, typically leading to "skeletal" ciliopathies such as Jeune asphyxiating thoracic dystrophy. Intriguingly, IFT gene mutations also cause eye, kidney and brain ciliopathies often linked to defects in the transition zone (TZ), a ciliary gate implicated in Hedgehog signalling. Here, we identify a C. elegans temperature-sensitive (ts) IFT-dynein mutant (che-3; human DYNC2H1) and use it to show a role for retrograde IFT in anterograde transport and ciliary maintenance. Unexpectedly, correct TZ assembly and gating function for periciliary proteins also require IFT-dynein. Using the reversibility of the novel ts-IFT-dynein, we show that restoring IFT in adults (post-developmentally) reverses defects in ciliary structure, TZ protein localisation and ciliary gating. Notably, this ability to reverse TZ defects declines as animals age. Together, our findings reveal a previously unknown role for IFT in TZ assembly in metazoans, providing new insights into the pathomechanism and potential phenotypic overlap between IFT- and TZ-associated ciliopathies.
机译：由纤毛功能障碍引起的肝细胞病患者遗传症遗传紊乱经常涉及肠道颗粒式（IFT）系统。 IFT亚基的突变 - 包括IFT-Dynein MotyNC2H1-Wheacair睫状体结构和刺猬信号，通常导致“骨骼”纤维病如Jeune窒息的胸营养不良症。有趣的是，IFT基因突变也造成眼睛，肾脏和脑纤维病经常与过渡区（TZ）中的缺陷连接，纤毛栅极涉及刺猬信号传导。在这里，我们鉴定C.杆状杆菌温度敏感（TS）IFT-Dynein突变体（Che-3;人Dync2H1），并用它来显示在逆行运输和睫状体维护中的逆行IFT的作用。意外地，术语蛋白质的正确TZ组件和门控功能也需要IFT- Dynein。利用新型TS-IFT-DYNIN的可逆性，我们表明IFT在成人中（发作后）逆转睫状体结构，TZ蛋白定位和睫状体门的缺陷。值得注意的是，这种反向TZ缺陷的能力随着动物年龄而下降。我们的研究结果在一起，揭示了在美洲达托纳的TZ组件中的IFT在TZ组件中的一个先前未知的作用，为IFT-和TZ相关的纤维病之间的潜在表型重叠提供了新的洞察力。

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