Since the cloning of ABCC1 in the early 90s [17,18,19], progress has been made towards establishing a functional relationship between ABCC1 and CFTR. For example, functional complementation of dysfunctional CFTR by ABCC1 following chemotherapy that resulted in increased expression of ABCC1 was associated with improved lung function in a CF patient [15]. Here, CFTR is linked to cystic fibrosis.