Joubert syndrome 26 protein enforces compartmentalized motility of a ciliary kinesin

• 成果类型: Article
• 署名作者: Wang, Shimin; Li, Ming; Chen, Guanghan; Chen, Zhe; Lei, Kexin; Okten, Zeynep; Xie, Shanshan; Zhou, Tianhua; Li, Wei; Ou, Guangshuo
• 署名单位: Tsinghua University; Tsinghua University; Tsinghua University; Tsinghua University; Tsinghua University; Technical University of Munich; Zhejiang University; Zhejiang University; Tsinghua University
• 刊物名称: PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA
• ISSN/ISSBN: 0027-8424; 1091-6490
• DOI: 10.1073/pnas.2504374122
• 发表日期: 2025-11-25
• 页码: e2504374122
• 关键词: cilia; kinesin-2; kinesin handover; intraflagellartransport (IFT); joubert syndrome; INTRAFLAGELLAR TRANSPORT MOTORS; mutations; microtubules; mutants; subunit; complex; lacking; IFT88; link

摘要

Cilia are essential cellular antennae that rely on precise motor- driven transport to assemelegans-cooperate to transport cargo along cilia, with kinesin- II operating in the middle segment and OSM- 3 taking over distally. However, how kinesin- II is spatially confined to prevent its invasion into distal regions remains unclear. Here, we identify JBTS- 26 localizes to axonemal doublet microtubules in the ciliary middle segment, distal transport. Loss of JBTS- 26 allows kinesin- II to invade the distal cilium and slows and link defective motor handover to ciliopathy pathogenesis.