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Phase separation of TTBK2 and CEP164 is necessary for ciliogenesis
Journal article   Peer reviewed

Phase separation of TTBK2 and CEP164 is necessary for ciliogenesis

Po-Chun Chou, Yu-Hao Lin, I-Hsuan Lin, Tzu-Ying Lin, Yun-Chia Huang, Shi-Rong Hong, Ting-Jui Ben Chang, Yin Fang, Yizhi Lin, T. Tony Yang, …
Cell reports (Cambridge), Vol.44(6), p.115810
24/06/2025
PMID: 40483689

Abstract

centriole distal appendages CEP164 ciliogenesis intrinsically disordered protein liquid-liquid phase separation TTBK2
The primary cilium plays a crucial function in sensing and transmitting extracellular signals into cells. The initiation of ciliogenesis hinges on the recruitment of Tau tubulin kinase 2 (TTBK2) to the distal appendages (DAs) of centrioles through CEP164. However, the detailed mechanism underlying the CEP164/TTBK2 interaction at DAs during ciliogenesis remains incompletely understood. In this study, we unveil that CEP164 features a long intrinsically disordered region and forms dynamic condensates with TTBK2 through phase separation. Our investigation demonstrates that CEP164 undergoes phase separation with TTBK2 through multivalent electrostatic interactions. These interactions facilitate the efficient recruitment of TTBK2 to DAs, thereby kickstarting the process of cilia formation. Therefore, our findings provide insights into the molecular regulation of CEP164/TTBK2 interactions at DAs and highlight the pivotal role of phase separation in promoting cilia formation. [Display omitted] •CEP164 forms dynamic, liquid-like condensates with TTBK2•Electrostatic interaction drives the phase separation of CEP164 and TTBK2•Phase separation of TTBK2 and CEP164 enhances TTBK2 recruitment to centrioles•Phase separation of TTBK2 and CEP164 is essential for efficient ciliogenesis Chou et al. demonstrate that CEP164 recruits TTBK2 to centriole distal appendages through phase separation driven by electrostatic interactions between their intrinsically disordered regions. This multivalent interaction forms dynamic condensates, facilitating efficient TTBK2 recruitment and initiating ciliogenesis, highlighting the critical role of phase separation in cilia formation.
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https://doi.org/10.1016/j.celrep.2025.115810View
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