RTI uses cookies to offer you the best experience online. By clicking “accept” on this website, you opt in and you agree to the use of cookies. If you would like to know more about how RTI uses cookies and how to manage them please view our Privacy Policy here. You can “opt out” or change your mind by visiting: http://optout.aboutads.info/. Click “accept” to agree.
Increased functional cell surface expression of CFTR and Delta F508-CFTR by the anthracycline doxorubicin
Maitra, R., Shaw, CM., Stanton, BA., & Hamilton, JW. (2001). Increased functional cell surface expression of CFTR and Delta F508-CFTR by the anthracycline doxorubicin. American Journal of Physiology - Cell Physiology, 280(5), C1031-C1037.
Cystic fibrosis (CF) is a disease that is caused by mutations within the cystic fibrosis transmembrane conductance regulator (CFTR) gene. The most common mutation, Delta F508, accounts for 70% of all CF alleles and results in a protein that is defective in folding and trafficking to the cell surface. However, Delta F508-CFTR is functional when properly localized. We report that a single, noncytotoxic dose of the anthracycline doxorubicin (Dox, 0.25 muM) significantly increased total cellular CFTR protein expression, cell surface CFTR protein expression, and CFTR-associated chloride secretion in cultured T84 epithelial cells. Dox treatment also increased Delta F508-CFTR cell surface expression and Delta F508-CFTR-associated chloride secretion in stably transfected Madin-Darby canine kidney cells. These results suggest that anthracycline analogs may be useful for the clinical treatment of CF.
