ENA0000GenomicsThe Scripps Research Institutehttps://www.ebi.ac.uk/ena/browser/view/PRJNA136507Homo sapiensOur laboratory has held a long interest in the glycosylation changes seen on the surface of airway epithelia of patients with the disease cystic fibrosis (CF). Experiments from our laboratory have detailed a CF glycosylation phenotype of increased Fuca1,3/4 and decreased Fuca1,2 and sialic acid on the surfaces of immortalized and primary CF cells compared to non-CF cells. Further, we have shown that gene transfer and subsequent expression of a wild type CF plasmid in CF airway cells results in correction or reversal of this glycosylation phenotype. We hypothesize that the changes in glycosylation seen in CF cells are key in the pathophysiology of the cystic fibrosis airway disease. For example, it has been shown that Pseudomonas aeruginosa, a bacterium that has a predilection for colonizing CF airways, adheres to asialylated glycolipids and glycoconjugates with terminal Fuca1,3/4. One focus of our laboratory is to elucidate the etiology of the glycosylation changes seen in CF cells and the mechanism by which these changes are reversed by wild type CFTR gene transfer. Overall design: We propose to study the gene expression of immortalized and primary CF and non-CF airway epithelial cells: 1. CF/T43 vs. BEAS-2B cells. These are two widely used immortalized airway cell lines that we have used extensively in the past. 2. C38 cells; C38 cells are IB3 cells expressing wtCFTR. The experimental focus is to elucidate the etiology of the glycosylation changes seen in Cystic Fibrosis (CF) cells and the mechanism by which these changes are reversed by wild type CFTR gene transfer. To do so, the gene expression of immortalized and primary CF and non-CF airway epithelial cells were compared and studied. Cell lines used were CF/T43 and BEAS-2B, both widely used immortalized airway cell lines. Other cell lines studied included C38 cell lines (clonal derivatives of IB3 cells expressing wtCFTR).ENATransitional Epithelial Cell, Transitional, Cystic fibrosis NOS, pseudomonas aeruginosa, Squamous Cells, Epithelial Cell, Glandular Epithelial Cell., susceptibility to chronic infection by, Meconium obstruction of intestine in mucoviscidosis, Mucoviscidosis, Adenomatous, Gene, Cystic fibrosis NOS (disorder), Pancreas Fibrocystic Diseases, Glandular Epithelial, Squamous, Glandular, Transitional Epithelial, Cell, CF - Cystic fibrosis, cystic fibrosis with pulmonary manifestations, Squamous Epithelial Cells, Cuboidal Glandular Epithelial Cells, tracheobronchial tree segment, CYSTIC FIBROS W/O ILEUS, cystic fibrosis with other manifestations (disorder), Columnar Glandular Epithelial Cells, cystic fibrosis lung disease, Pancreatic, Fibrosis, modifier of, Meconium ileus in cystic fibrosis (disorder), Squamous Epithelial, Pulmonary, Epithelial, in cystic fibrosis, Adenomatous Epithelial Cell, Cystic fibrosis (disorder), Adenomatous Epithelial Cells, Pancreatic Cystic Fibrosis, Squamous Cell, Gene Expressions, cystic fibrosis with meconium ileus, airways, Cystic Fibrosis of Pancreas, cystic fibrosis with other manifestations, Cystic fibrosis without mention of meconium ileus, Glandular Epithelial Cells, segment of tracheobronchial tree, cystic fibrosis with combined manifestations, cystic fibrosis, Adenomatous Epithelial, cystic fibrosis with meconium ileus (disorder), Expressions, cystic fibrosis with gastrointestinal manifestations, respiratory conducting tube, Fibrocystic Disease of Pancreas, Fibrocystic disease, Cystic, CF, Cystic Fibrosis, airway, Epithelial Cells, mucoviscidosis, Transitional Epithelial Cells, Pancreas Fibrocystic Disease, Cells, Pulmonary Cystic Fibrosis, Expression, Squamous Epithelial Cell, cystic fibrosis with pulmonary manifestations (disorder)human being, human., man0.00.00.00.00.00falseHomo sapiensGene expression of immortalized cystic fibrosis and non-cystic fibrosis airway epithelial cells2022-05-122014-02-11PRJNA136507GSE264829606