Investigation of the cell type, intracellular organelle or macromolecule that functions abnormally in Cystic Fibrosis suffers from lack of tissue to study. No animal model of CF exists at present for experimentation. Because epithelial cells selectively express the genetic defect in CF patients, the goal of obtaining a cell line with the CF defect for study in culture was undertaken. We have been successful in isolating urinary epithelial cells from normal and CF newborn babies and, in addition, have developed primary cultures of epithelial cells from minor salivary and submandibular glands. Further efforts are proposed to improve the growth of these cell cultures as well as to initiate epithelial cell cultures from sweat gland, lung and colon. The epithelial cell cultures so far established will be utilized to test the hypothesis that CF results from a defect in ion transport. Studies to assess this hypothesis will include measurement of potential difference, sodium and chloride flux, histochemistry, cytochemistry, electron spin resonance and the production of CF factors.
