The most common CF mutation, the deletion of phenylalanine 508 (F508del), is responsible for two distinct defects: 1) a processing defect, that is, misfolding of the F508del-CFTR protein, which causes its retention at the endoplasmic reticulum (ER) and premature degradation by the ubiquitin/proteasome system7, 8, 9, 10, 11; and 2) a channel gating defect due to abnormal persistence of a closed state12, 13, 14, 15. Here, CFTR is linked to cystic fibrosis.