Epithelial cells form a semi-permeable barrier between the body and the environment.
The ability of these cells to selectively absorb or secrete inorganic and organic ions into externalized compartments determines the health of the organism. My laboratory's goal is to understand why ion-transport defects cause CF disease and to develop treatments based on compensating for the abnormal pathways. Our diverse experimental approaches to these questions include biochemical and biophysical studies of purified chloride channels, electrical recordings from single cells, and in vivo and in vitro studies of animal models of disease. Examination of mouse models for CF, revealed the potential for secondary genes to modify the outcome of the disease. We are using electrophysiological techniques to determine the function of these genes in modifying epithelial cell ion transport.