The ΔF508 mutation is a three-base pair deletion in the cystic fibrosis (CF) transmembrane conductance regulator (CFTR) found in one out of 30 Caucasians and is the leading cause of CF. These growth-enhanced cell lines were created by introducing Bmi-1, a protooncogene that maintains stem cells, and hTERT, the catalytic subunit of telomerase, into three non-CF and three CF human bronchial epithelial cell preparations. The cell line may be used to evaluate how the molecular mechanisms which are responsible for CF result in clinical symptoms and to test pharmacological and gene therapy agents for efficacy in treating CF lung disease.
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- Novel human bronchial epithelial cell lines for cystic fibrosis research Am J Physiol Lung Cell Mol Physiol 2009 Jan;296(1):L82-91