Cystic fibrosis (CF) is a genetic disease caused by mutations in a protein called CFTR. This protein controls the movement of salt and water into and out of cells throughout the body. The protein does not work properly in people with cystic fibrosis. Although this typically causes serious lung disease, the protein is also involved in the release of insulin from the beta cells. This can lead to a form of diabetes called cystic fibrosis-related diabetes (CFRD) in which the beta cells do not produce enough insulin.
Research has shown that new treatments called CFTR modulators can improve the function of this faulty protein. Although these treatments were designed to improve lung function, they also can improve the release of insulin from the pancreas. These drugs can also improve digestion and nutrition by reducing inflammation within the part of the pancreas that is involved in digesting food.
These new CFTR modulators therefore represent a promising advance for CFRD. Although they are not a cure, if started early they can simplify the management of diabetes and possibly prevent it in people with cystic fibrosis.
About the author
Yunhua Ren
Yunhua is a grad student at University of Toronto. Yunhua's research focuses on the mechanisms underlying cystic fibrosis–related diabetes (CFRD), with a particular interest in how pancreatic ductal epithelial cell (PDEC) dysfunction affects islet biology. Yunhua's work uses human pancreas slice models and organoid systems to investigate how CFTR deficiency alters intercellular signaling and impairs insulin secretion.