Cystic fibrosis transmembrane conductance regulator（CFTR）is an ATP-gated，cAMP-dependent chloride channel. The basic biophysical and pathological functions of CFTR are related with the secretion of chloride ion in epithelial cells and tissues. Mutations in CFTR cause cystic fibrosis（CF），which is a rare but fatal autosomal recessive inheritant disease，mainly affecting glan? dular epithelial function in respiratory tract，intestinal and reproductive system. New drugs targeting human CFTR gene mutations have been developed to significantly prolong lifeand improve respiratory symptoms of CF patients. Recent evidence suggested that CFTR plays a functional role in vasoconstriction and the formation of myocardial action potential. As a channel protein，CFTR may al? so functions as a multiprotein/channel complex，which has been demonstrated in the development of cardiovascular diseases such as myocardial ischemia and pulmonary hypertension.