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Scientific article
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English

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

Contributors

El Zein, Loubna; Ait-Lounis, Aouatef; Morlé, Laurette; Thomas, Joëlle; Chhin, Brigitte; Spassky, Nathalie; Reith, Walter; Durand, Bénédicte

Published in

Journal of cell science, vol. 122, no. Pt 17, p. 3180-3189

Publication date

2009

Abstract

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.

Keywords

  • Amino Acid Sequence
  • Animals
  • Cilia/chemistry/physiology
  • Ciliary Motility Disorders/genetics/metabolism
  • DNA-Binding Proteins/chemistry/genetics/metabolism
  • Gene Expression Regulation, Developmental
  • Humans
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Molecular Sequence Data
  • Protein Binding
  • Sequence Alignment
  • Transcription Factors/chemistry/genetics/metabolism

UNIGE affiliation entities

UNIGE research groups

Citation (ISO format)

EL ZEIN, Loubna et al. RFX3 governs growth and beating efficiency of motile cilia in mouse and controls the expression of genes involved in human ciliopathies. In: Journal of cell science, 2009, vol. 122, n° Pt 17, p. 3180–3189. doi: 10.1242/jcs.048348

Main files (7)

Article (Published version)
Article (Published version)
Article (Published version)
Article (Published version)
Article (Published version)
Article (Published version)
Article (Published version)

Identifiers

Additional URL for this publication

http://jcs.biologists.org/cgi/content/full/122/17/3180

Journal ISSN

0021-9533
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2810downloads

Technical informations

Creation

30/03/2010 15:19:00

First validation

30/03/2010 15:19:00

Update

14/03/2023 15:26:03

Status update

14/03/2023 15:26:03

Last indexation

29/10/2024 14:19:47
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