Some argue that accumulated misfolded CFTR proteins in the endoplasmic reticulum (ER) lead to inflammatory responses via an unfolded protein response (UPR) [6]; others have focused on the possible intrinsic effects of a mutant CFTR, not only on the airway epithelial cells but also on the immune system for CF hyperinflammation [7,8]. Here, CFTR is linked to cystic fibrosis.