Ciliary transition zone proteins coordinate ciliary protein composition and ectosome shedding.
Ciliary transition zone proteins coordinate ciliary protein composition and ectosome shedding.
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纤毛过渡区蛋白协调纤毛蛋白组成和胞外体脱落
DOI：
10.1038/s41467-022-31751-0
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发表时间：
2022-07-09
影响因子：
16.6
通讯作者：
中科院分区：
文献类型：
--
作者：
纤毛/鞭毛的过渡区（TZ）作为控制纤毛蛋白进入/退出的扩散屏障。TZ蛋白的突变破坏屏障功能并导致多种人类疾病。然而，系统的纤毛组成和信号相关的过程中，不同的TZ蛋白的调节还没有完全理解。在这里，我们揭示了TCTN 1在莱茵衣藻中的损失破坏了TZ中楔形结构的组装。从野生型和三个TZ突变体，tctn 1，cep 290和nphp 4纤毛蛋白质组学分析，显示了独特的作用，每个TZ亚基在纤毛组成的调节，解释不同的TZ突变体的表型多样性。有趣的是，我们发现TZ的缺陷损害了纤毛外体的形成和生物活性。总的来说，我们的研究结果提供了系统的见解纤毛组成的TZ蛋白的调节，并揭示了TZ和纤毛外体之间的联系。纤毛从细胞伸出以提供感觉功能，纤毛中断可导致称为纤毛病的多种疾病。在这里，作者表明，睫状体病基因TCTN 1的功能，以调节纤毛过渡区和外泌体的形成。
The transition zone (TZ) of the cilium/flagellum serves as a diffusion barrier that controls the entry/exit of ciliary proteins. Mutations of the TZ proteins disrupt barrier function and lead to multiple human diseases. However, the systematic regulation of ciliary composition and signaling-related processes by different TZ proteins is not completely understood. Here, we reveal that loss of TCTN1 in Chlamydomonas reinhardtii disrupts the assembly of wedge-shaped structures in the TZ. Proteomic analysis of cilia from WT and three TZ mutants, tctn1, cep290, and nphp4, shows a unique role of each TZ subunit in the regulation of ciliary composition, explaining the phenotypic diversity of different TZ mutants. Interestingly, we find that defects in the TZ impair the formation and biological activity of ciliary ectosomes. Collectively, our findings provide systematic insights into the regulation of ciliary composition by TZ proteins and reveal a link between the TZ and ciliary ectosomes. Cilia project from cells to serve sensory functions, and ciliary disruption can result in multiple disorders known as ciliopathies. Here the authors show that the ciliopathy gene TCTN1 functions to regulate the ciliary transition zone and ectosome formation.
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影响因子：
9.2
作者：
Long, Huan;Zhang, Fan;Xu, Nannan;Liu, Gai;Diener, Dennis R.;Rosenbaum, Joel L.;Huang, Kaiyao
通讯作者：
Huang, Kaiyao
影响因子：
9.8
作者：
Li C;Jensen VL;Park K;Kennedy J;Garcia-Gonzalo FR;Romani M;De Mori R;Bruel AL;Gaillard D;Doray B;Lopez E;Rivière JB;Faivre L;Thauvin-Robinet C;Reiter JF;Blacque OE;Valente EM;Leroux MR
通讯作者：
Leroux MR
影响因子：
7.7
作者：
Li S;Wan KY;Chen W;Tao H;Liang X;Pan J
通讯作者：
Pan J
影响因子：
9.8
作者：
Gogendeau, Delphine;Lemullois, Michel;Tassin, Anne-Marie
通讯作者：
Tassin, Anne-Marie
影响因子：
21.3
作者：
Chih, Ben;Liu, Peter;Peterson, Andrew S.
通讯作者：
Peterson, Andrew S.