Immunolocalization of CFTR in Intact Tissue and Cultured Cells

Cloning of the cystic fibrosis (CF) gene provided, for the first time, the structural information needed to more precisely define the CF defect (1 -3 ). This genetic information was used to develop powerful molecular and antibody reagents that helped define cystic fibrosis transmembrane conductance regulator (CFTR) structure-function relationships and characterize CFTR expression throughout the body. This chapter focuses on how genetic information can be used to develop antipeptide antibodies and how these antibodies can be used to immunolocalize proteins in intact and cultured cells. By identifying which cells expressed CFTR, and correlating that information with existing knowledge of the physiology of those cells, the role of CFTR in normal cell function and the pathophysiology of CF was elucidated.