The transcription factor RFX3 directs nodal cilium development and left-right asymmetry specification

Autor: Bénédicte Durand, Aouatef Ait-Lounis, Catherine Ucla, Frédéric Flamant, Elsa Bonnafé, Walter Reith, J. Collignon, M. Touka, Dominique Baas, A. Moreau, Pierre Couble, Emmanuèle Barras, Raphaëlle Dubruille
Přispěvatelé: ProdInra, Migration, Centre de génétique et de physiologie moléculaire et cellulaire (CGPhiMC), Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon, Unité mixte de recherche biologie moléculaire de la cellule, École normale supérieure - Lyon (ENS Lyon)-Institut National de la Recherche Agronomique (INRA)-Centre National de la Recherche Scientifique (CNRS), École normale supérieure de Lyon (ENS de Lyon)-Institut National de la Recherche Agronomique (INRA)-Centre National de la Recherche Scientifique (CNRS)
Předmět:
Fetal Death/genetics
Male
[SDV]Life Sciences [q-bio]
DNA-Binding Proteins/deficiency/genetics/ physiology
ddc:616.07
MESH: Base Sequence
MESH: Mice
Knockout
Dyneins/genetics
Mice
0302 clinical medicine
MESH: Gene Expression Regulation
Developmental
Mammalian Genetic Models with Minimal or Complex Phenotypes
MESH: Animals
MESH: In Situ Hybridization
Fluorescence
In Situ Hybridization
Fluorescence
Mice
Knockout
Genetics
Regulation of gene expression
0303 health sciences
Cilium
MESH: DNA
Gene Expression Regulation
Developmental
MESH: Transcription Factors
Cell biology
[SDV] Life Sciences [q-bio]
DNA-Binding Proteins
Female
Regulatory Factor X1
SOURIS TRANSGENIQUE
MESH: Body Patterning
MESH: Microscopy
Electron
Scanning
Body Patterning/genetics/ physiology
Dynein
Regulatory Factor X Transcription Factors
[SDV.BC]Life Sciences [q-bio]/Cellular Biology
DNA/genetics
[INFO] Computer Science [cs]
Biology
03 medical and health sciences
MESH: Mice
Inbred C57BL
MESH: Cilia
Intraflagellar transport
BIOLOGIE DU DEVELOPPEMENT
Animals
[INFO]Computer Science [cs]
Cilia
Transcription Factors/deficiency/genetics/ physiology
MESH: Mice
Fetal Death
Molecular Biology
Process (anatomy)
Transcription factor
Body Patterning
030304 developmental biology
Base Sequence
Dyneins
DNA
Cell Biology
GENETIQUE
Cilia/ ultrastructure
MESH: Male
MESH: Dynein ATPase
Mice
Inbred C57BL
Microscopy
Electron
Scanning
MESH: Fetal Death
RFX3
NODAL
MESH: Female
MESH: DNA-Binding Proteins
030217 neurology & neurosurgery
Transcription Factors
Zdroj: HAL
Molecular and Cellular Biology
Molecular and Cellular Biology, American Society for Microbiology, 2004, 24 (10), pp.4417-27
Molecular and Cellular Biology, American Society for Microbiology, 2004, pp.44717-4427
Molecular and Cellular Biology, American Society for Microbiology, 2004, 24 (10), pp.4417-4427
Molecular and Cellular Biology, Vol. 24, No 10 (2004) pp. 4417-4427
Molecular and Cellular Biology, 2004, 24 (10), pp.4417-4427
ISSN: 0270-7306
1098-5549
Popis: International audience; There are five members of the RFX family of transcription factors in mammals. While RFX5 plays a well-defined role in the immune system, the functions of RFX1 to RFX4 remain largely unknown. We have generated mice with a deletion of the Rfx3 gene. RFX3-deficient mice exhibit frequent left-right (LR) asymmetry defects leading to a high rate of embryonic lethality and situs inversus in surviving adults. In vertebrates, specification of the LR body axis is controlled by monocilia in the embryonic node, and defects in nodal cilia consequently result in abnormal LR patterning. Consistent with this, Rfx3 is expressed in ciliated cells of the node and RFX3-deficient mice exhibit a pronounced defect in nodal cilia. In contrast to the case for wild-type embryos, for which we document for the first time a twofold increase in the length of nodal cilia during development, the cilia are present but remain markedly stunted in mutant embryos. Finally, we show that RFX3 regulates the expression of D2lic, the mouse orthologue of a Caenorhabditis elegans gene that is implicated in intraflagellar transport, a process required for the assembly and maintenance of cilia. In conclusion, RFX3 is essential for the differentiation of nodal monocilia and hence for LR body axis determination.
Databáze: OpenAIRE
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