Polarity Proteins Control Ciliogenesis via Kinesin Motor Interactions

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• 作者：Shuling Fan ; Toby W. Hurd ; Chia-Jen Liu ; Samuel W. Straight ; Thomas Weimbs ; Elizabeth A. Hurd ; Steven E. Domino ; Ben Margolis
• 关键词：CELLBIO ; DNA ; RNA
• 刊名：Current Biology
• 出版年：2004
• 出版时间：24 August 2004
• 年：2004
• 卷：14
• 期：16
• 页码：1451-1461
• 全文大小：773 K

文摘

Background: Cilia are specialized organelles that play a fundamental role in several mammalian processes including left-right axis determination, sperm motility, and photoreceptor maintenance. Mutations in cilia-localized proteins have been linked to human diseases including cystic kidney disease and retinitis pigmentosa. Retinitis pigmentosa can be caused by loss-of-function mutations in the polarity protein Crumbs1 (CRB1), but the exact role of CRB1 in retinal function is unclear.

Results: Here we show that CRB3, a CRB1-related protein found in epithelia, is localized to cilia and required for proper cilia formation. We also find that the Crumbs-associated Par3/Par6/aPKC polarity cassette localizes to cilia and regulates ciliogenesis. In addition, there appears to be an important role for the polarity-regulating 14-3-3 proteins in this process. Finally, we can demonstrate association of these polarity proteins with microtubules and the microtubular motor KIF3/Kinesin-II.

Conclusions: Our findings point to a heretofore unappreciated role for polarity proteins in cilia formation and provide a potentially unique insight into the pathogenesis of human kidney and retinal disease.