It is now over 25 years since the causative gene in cystic fibrosis (CF) was identified as ABCC7 [also known as the CF transmembrane conductance regulator (CFTR)] [29] and a huge wealth of information has been obtained for this atypical ABC protein which is not in fact a primary active transporter but an ATP-gated chloride channel. This evidence concerns the gene CFTR and cystic fibrosis.