Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents

This Article Full Text Full Text (PDF, 3147K) PDF+supp data (5042K) PPT slides of all figures Supplemental Material Index Alert me when this article is cited Citation Map Services Email this article Similar articles in this journal Similar articles in PubMed Alert me to new content in the JCB Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via CrossRef Citing Articles via Google Scholar Google Scholar Articles by Voronina, V. A. Articles by Moon, R. T. Search for Related Content PubMed PubMed Citation Articles by Voronina, V. A. Articles by Moon, R. T. Social Bookmarking                            What's this?

Inactivation of Chibby affects function of motile airway cilia

¹ Department of Pharmacology, ² Department of Comparative Medicine, ³ Department of Immunology, ⁴ Howard Hughes Medical Institute, and ⁵ Institute for Stem Cell and Regenerative Medicine, University of Washington, Seattle, WA 98195
⁶ Cystic Fibrosis Pulmonary Research and Treatment Center, Department of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599
⁷ Department of Pharmacological Sciences, State University of New York at Stony Brook, Stony Brook, NY 11794

Correspondence to Ken-Ichi Takemaru: takemaru{at}pharm.stonybrook.edu; or Randall T. Moon rtmoon{at}u.washington.edu

Chibby (Cby) is a conserved component of the Wnt–β-catenin pathway. Cby physically interacts with β-catenin to repress its activation of transcription. To elucidate the function of Cby in vertebrates, we generated Cby^–/– mice and found that after 2–3 d of weight loss, the majority of mice die before or around weaning. All Cby^–/– mice develop rhinitis and sinusitis. When challenged with Pseudomonas aeruginosa isolates, Cby^–/– mice are unable to clear the bacteria from the nasal cavity. Notably, Cby^–/– mice exhibit a complete absence of mucociliary transport caused by a marked paucity of motile cilia in the nasal epithelium. Moreover, ultrastructural experiments reveal impaired basal body docking to the apical surface of multiciliated cells. In support of these phenotypes, endogenous Cby protein is localized at the base of cilia. As the phenotypes of Cby^–/– mice bear striking similarities to primary ciliary dyskinesia, Cby^–/– mice may prove to be a useful model for this condition.

Abbreviations used in this paper: BAC, bacterial artificial chromosome; Cby, Chibby; CF, cystic fibrosis; CFU, colony-forming unit; LB, Luria broth; MCT, mucociliary transport; PCD, primary ciliary dyskinesia; PMEF, primary mouse embryonic fibroblast; TEM, transmission EM.

© 2009 Voronina et al.
This article is distributed under the terms of an Attribution–Noncommercial–Share Alike–No Mirror Sites license for the first six months after the publication date (see http://www.jcb.org/misc/terms.shtml). After six months it is available under a Creative Commons License (Attribution–Noncommercial–Share Alike 3.0 Unported license, as described at http://creativecommons.org/licenses/by-nc-sa/3.0/).

CiteULike

Complore

Connotea

Del.icio.us

Digg

Facebook

Reddit

Technorati

Twitter

This article has been cited by other articles:

• Gu, B., Sun, P., Yuan, Y., Moraes, R. C., Li, A., Teng, A., Agrawal, A., Rheaume, C., Bilanchone, V., Veltmaat, J. M., Takemaru, K.-I., Millar, S., Lee, E. Y.-H.P., Lewis, M. T., Li, B., Dai, X. (2009). Pygo2 expands mammary progenitor cells by facilitating histone H3 K4 methylation. JCB 185: 811-826 [Abstract] [Full Text]  

Home | Help | Feedback | Subscriptions | Archive | Search | Table of Contents