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Project properties
| Title | Transcriptional regulation of CFTR gene expression |
|---|---|
| Keywords | cystic fibrosis nuclear receptors transcriptional regulation |
| Researchers |
dr. J.W. Jonker prof. dr. H.J. Verkade |
| Nature of the research | This is a fundamental research project using various molecular and cell biological techniques aimed at unraveling the molecular mechanisms underlying the pathophysiology of cystic fibrosis |
| Fields of study | pediatrics medical biology molecular biology |
| Background / introduction |
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| Cystic fibrosis (CF) is the most common genetic disease amongst the Caucasian population with a frequency ~1:3,000 live births and the clinical course is dominated by lung disease and recurrent pulmonary bacterial infections. Major improvements in treatment of Cystic fibrosis (CF), which include proactive treatment of airway infections and nutritional/lifestyle management have increased average life expectancy to about 40 years from about 4 years in the 1950s. |
| Research question / problem definition |
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| The aim of this project is to investigate the regulation of the CFTR gene by the Nuclear Hormone Receptor (NHR) family of transcription factors. As NHRs are ligand modulated transcription factors, knowledge of the complete regulatory matrix is expected to reveal new therapeutically accessible pathways for the treatment of cystic fibrosis. |
| Workplan |
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Our lab uses functional genetic screening techniques to identify transcription factors involved in the regulation of vatious diseases including Metabolic Diseases, Cancer and Cystic Fibrosis. Genetic, pharmacologic and metabolic approaches will be utilized to delineate the molecular mechanisms and the progression of diesease. Techniques/approaches that will be used include mouse models, cell culture, molecular biology and RNAi. |
| References |
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Jonker, J.W., et al. (2012) A PPARγ-FGF1 axis is required for adipose remodelling and metabolic homeostasis. Nature. 485, 391-394. Lamia, K.A., et al. (2011) Cryptochromes mediate rhythmic repression of the glucocorticoid receptor. Nature. 480, 552-556. |
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