The etiology of CFRD could thus both be due to damages on theexocrine pancreas with an indirect effect on pancreatic islets; and/or by anintrinsic islet defect through the presence of CFTR in the islet cells.16 In this review, we will seek to assess these 2 hypotheses from a cellbiological and molecular perspective and give special consideration to how intrinsiceffects of CFTR mutations could lead to the altered hormone secretion by β- andα-cells seen in CFRD patients. Here, CFTR is linked to cystic fibrosis-related diabetes.