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First published online 11 August 2009
doi: 10.1242/jcs.048348

This Article Figures Only Full Text Full Text (PDF) Supplementary Material All Versions of this Article: jcs.048348v1 122/17/3180    most recent Alert me when this article is cited Alert me if a correction is posted Services Email this article to a friend Related articles in JCS Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by El Zein, L. Articles by Durand, B. Search for Related Content PubMed PubMed Citation Articles by El Zein, L. Articles by Durand, B. Pubmed/NCBI databases Gene GEO Profiles HomoloGene Protein UniGene Social Bookmarking                         What's this?

RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies

¹ Université de Lyon, Lyon, F-69003, Université Lyon 1, CNRS, UMR5534, CGMC, Centre de Génétique Moléculaire et Cellulaire, Villeurbanne, F-69622, France
² Department of Pathology and Immunology, Faculty of Medicine, University of Geneva, CMU, 1 rue Michel-Servet, CH-1211 Geneva, Switzerland
³ INSERM U711, Hôpital Salpêtrière, 47 Boulevard de l'hôpital, 75013 Paris, France

^* Author for correspondence (durand-b{at}univ-lyon1.fr)

Accepted 21 June 2009

Cilia are cellular organelles that play essential physiological and developmental functions in various organisms. They can be classified into two categories, primary cilia and motile cilia, on the basis of their axonemal architecture. Regulatory factor X (RFX) transcription factors have been shown to be involved in the assembly of primary cilia in Caenorhabditis elegans, Drosophila and mice. Here, we have taken advantage of a novel primary-cell culture system derived from mouse brain to show that RFX3 is also necessary for biogenesis of motile cilia. We found that the growth and beating efficiencies of motile cilia are impaired in multiciliated Rfx3^–/– cells. RFX3 was required for optimal expression of the FOXJ1 transcription factor, a key player in the differentiation program of motile cilia. Furthermore, we demonstrate for the first time that RFX3 regulates the expression of axonemal dyneins involved in ciliary motility by binding directly to the promoters of their genes. In conclusion, RFX proteins not only regulate genes involved in ciliary assembly, but also genes that are involved in ciliary motility and that are associated with ciliopathies such as primary ciliary dyskinesia in humans.

Key words: Axonemal dyneins, Cilia, Mouse primary cell cultures, Primary ciliary dyskinesia, RFX proteins

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				B. P. Piasecki, J. Burghoorn, and P. Swoboda Regulatory Factor X (RFX)-mediated transcriptional rewiring of ciliary genes in animals PNAS, July 20, 2010; 107(29): 12969 - 12974. [Abstract] [Full Text] [PDF]