ABSTRACT
I. Introduction Cystic fibrosis (CF) is a disease of abnormal ion transport. Specifically, abnormalities in the expression and function of the cystic fibrosis transmembrane conductance regulator (CFTR) result in abnormal salt and water transport across epithelial surfaces in the gastrointestinal and hepatobiliary systems, respiratory tract, reproductive system, and sweat glands. With the exception of the sweat glands, abnormal salt and water transport eventuate in end-organ damage causing significant morbidity and severely shortening life span.
