ABSTRACT
Defective cystic brosis transmembrane conductance regulator (CFTR) function produces abnormal epithelial chloride (Cl−) and sodium (Na+) ion movement, which produces a characteristic, measurable potential dierence (PD) across cell membranes and allow a direct and dynamic assessment of ion channel function. As a bioelectric assay of CFTR function, PD has developed as a clinical trial outcome particularly for therapies targeting the CFTR gene or protein, where activity of the protein is interrogated over and above the more standard and surrogate cystic brosis (CF) indices.
