Structure Makes a Difference: IFT Complex in Ciliary Function and Ciliopathy

Ying Liu

doi:10.1002/cm.70033

2025-09-01

DOI：10.1002/cm.70033

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摘要

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纤毛是进化上保守的真核细胞表面细胞器，其组装、维持和信号转导依赖于IFT（ intraflagellar transport，纤毛内运输）系统。IFT系统通过蛋白质复合物和分子马达的协同作用，协调结构成分和信号分子的双向运输。IFT复合物在纤毛基部组装成anterograde trains（顺行列车），并在纤毛尖端经历结构重塑形成retrograde trains（逆行列车），其双向运动性受列车自身及微管轨道上的修饰调控。BBSome随IFT列车运行，作为关键适配器，主要将膜货物连接至IFT列车以供货物退出纤毛。人类纤毛病中与纤毛相关的基因突变有助于增进对IFT机器的理解。本文全面阐述了IFT复合物的分子结构、运输机制和调控网络，将它们的功能失调与疾病表型联系起来，并推进了机制层面的认识。

Keyword:

anterograde train

cilia

ciliopathy

intraflagellar transport (IFT)

retrograde train

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