Human Lymphocytes Transcribe the Cystic Fibrosis Transmembrane Conductance Regulator Gene and Exhibit CF-defective CAMP-

Cystic fibrosis (CF) is the most common lethal genetic disease among Caucasians, primarily affecting epithe-lial tissues of the lung and gut. Mutations in a single gene, the cystic fibrosis transmembrane conductance regulator (CFTR), are responsible for this disease. Whether a physiological defect exists in the immune system of CF patients has remained controversial. A chloride ion transport defect has been described in human CF-derived lymphocytes; however, it has not been possible to detect CFTR mRNA in lymphocytes. We report here that normal human B-lymphoblasts display whole cell C1- conductances induced by cal-cium-mediated pathways, volume regulation, and cAMP which are equivalent to currents described in epithelial cells. B-lymphoblasts from CF-affected hu