16-P006 The ciliogenic transcription factor RFX3 is required for lung development in mice
نویسندگان
چکیده
We have developed anti-Evc2 antibodies and show that Evc2 and Evc co-localise at the basal body and also in primary cilia. In transfected cells, basal body and cilia localisation are only observed when Evc and Evc2 constructs are co-transfected but do not occur when either construct is transfected individually indicating that this localisation is co-dependent. Although Evc localises to cilia it is not essential for ciliogenesis; chondrocyte cilia from mice lacking Evc appear structurally normal on transmission electron microscopy. Finally, we have demonstrated that Evc and Evc2 are cilia transmembrane proteins by immunostaining in permeabilised and non-permeabilised cells, the C-terminus for both being intracellular and Evc2, but not Evc, having an extracellular portion.
منابع مشابه
Novel function of the ciliogenic transcription factor RFX3 in development of the endocrine pancreas.
The transcription factor regulatory factor X (RFX)-3 regulates the expression of genes required for the growth and function of cilia. We show here that mouse RFX3 is expressed in developing and mature pancreatic endocrine cells during embryogenesis and in adults. RFX3 expression already is evident in early Ngn3-positive progenitors and is maintained in all major pancreatic endocrine cell lineag...
متن کاملThe Ciliogenic Transcription Factor RFX3 Regulates Early Midline Distribution of Guidepost Neurons Required for Corpus Callosum Development
The corpus callosum (CC) is the major commissure that bridges the cerebral hemispheres. Agenesis of the CC is associated with human ciliopathies, but the origin of this default is unclear. Regulatory Factor X3 (RFX3) is a transcription factor involved in the control of ciliogenesis, and Rfx3-deficient mice show several hallmarks of ciliopathies including left-right asymmetry defects and hydroce...
متن کاملThe ciliogenic transcription factor Rfx3 is required for the formation of the thalamocortical tract by regulating the patterning of prethalamus and ventral telencephalon.
Primary cilia are complex subcellular structures that play key roles during embryogenesis by controlling the cellular response to several signaling pathways. Defects in the function and/or structure of primary cilia underlie a large number of human syndromes collectively referred to as ciliopathies. Often, ciliopathies are associated with mental retardation (MR) and malformation of the corpus c...
متن کاملThe Transcription Factor Rfx3 Regulates β-Cell Differentiation, Function, and Glucokinase Expression
OBJECTIVE Pancreatic islets of perinatal mice lacking the transcription factor Rfx3 exhibit a marked reduction in insulin-producing beta-cells. The objective of this work was to unravel the cellular and molecular mechanisms underlying this deficiency. RESEARCH DESIGN AND METHODS Immunofluorescence studies and quantitative RT-PCR experiments were used to study the emergence of insulin-positive...
متن کاملThe ciliogenic transcription factor Rfx3 is required for the formation of the thalamocortical tract by regulating patterning of prethalamus and ventral telencephalon
Primary cilia are complex subcellular structures that play key roles during embryogenesis by controlling the cellular response to several signaling pathways. Defects in the function and/or structure of primary cilia underlie a large number of human syndromes collectively referred to as ciliopathies. Often, ciliopathies are associated with mental retardation (MR) and malformation of the corpus c...
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ورودعنوان ژورنال:
- Mechanisms of Development
دوره 126 شماره
صفحات -
تاریخ انتشار 2009