author_facet El Zein, Loubna
Ait-Lounis, Aouatef
Morlé, Laurette
Thomas, Joëlle
Chhin, Brigitte
Spassky, Nathalie
Reith, Walter
Durand, Bénédicte
El Zein, Loubna
Ait-Lounis, Aouatef
Morlé, Laurette
Thomas, Joëlle
Chhin, Brigitte
Spassky, Nathalie
Reith, Walter
Durand, Bénédicte
author El Zein, Loubna
Ait-Lounis, Aouatef
Morlé, Laurette
Thomas, Joëlle
Chhin, Brigitte
Spassky, Nathalie
Reith, Walter
Durand, Bénédicte
spellingShingle El Zein, Loubna
Ait-Lounis, Aouatef
Morlé, Laurette
Thomas, Joëlle
Chhin, Brigitte
Spassky, Nathalie
Reith, Walter
Durand, Bénédicte
Journal of Cell Science
RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
Cell Biology
author_sort el zein, loubna
spelling El Zein, Loubna Ait-Lounis, Aouatef Morlé, Laurette Thomas, Joëlle Chhin, Brigitte Spassky, Nathalie Reith, Walter Durand, Bénédicte 1477-9137 0021-9533 The Company of Biologists Cell Biology http://dx.doi.org/10.1242/jcs.048348 <jats:p>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.</jats:p> RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies Journal of Cell Science
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title RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_unstemmed RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_full RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_fullStr RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_full_unstemmed RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_short RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_sort rfx3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
topic Cell Biology
url http://dx.doi.org/10.1242/jcs.048348
publishDate 2009
physical 3180-3189
description <jats:p>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.</jats:p>
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author El Zein, Loubna, Ait-Lounis, Aouatef, Morlé, Laurette, Thomas, Joëlle, Chhin, Brigitte, Spassky, Nathalie, Reith, Walter, Durand, Bénédicte
author_facet El Zein, Loubna, Ait-Lounis, Aouatef, Morlé, Laurette, Thomas, Joëlle, Chhin, Brigitte, Spassky, Nathalie, Reith, Walter, Durand, Bénédicte, El Zein, Loubna, Ait-Lounis, Aouatef, Morlé, Laurette, Thomas, Joëlle, Chhin, Brigitte, Spassky, Nathalie, Reith, Walter, Durand, Bénédicte
author_sort el zein, loubna
container_issue 17
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container_title Journal of Cell Science
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description <jats:p>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.</jats:p>
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spelling El Zein, Loubna Ait-Lounis, Aouatef Morlé, Laurette Thomas, Joëlle Chhin, Brigitte Spassky, Nathalie Reith, Walter Durand, Bénédicte 1477-9137 0021-9533 The Company of Biologists Cell Biology http://dx.doi.org/10.1242/jcs.048348 <jats:p>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.</jats:p> RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies Journal of Cell Science
spellingShingle El Zein, Loubna, Ait-Lounis, Aouatef, Morlé, Laurette, Thomas, Joëlle, Chhin, Brigitte, Spassky, Nathalie, Reith, Walter, Durand, Bénédicte, Journal of Cell Science, RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies, Cell Biology
title RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_full RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_fullStr RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_full_unstemmed RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_short RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_sort rfx3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
title_unstemmed RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies
topic Cell Biology
url http://dx.doi.org/10.1242/jcs.048348