CFTR expression regulation by the unfolded protein response

R Bartoszewski, A Rab, L Fu, S Bartoszewska… - Methods in …, 2011 - Elsevier
The cystic fibrosis transmembrane conductance regulator (CFTR) is a chloride channel and
key regulator of epithelial functions. Mutations in the CFTR gene lead to reduced or
dysfunctional CFTR protein and cause cystic fibrosis (CF), a generalized exocrinopathy
affecting multiple organs. In the airways, loss of CFTR function leads to thickened mucus,
reduced mucociliary clearance, chronic infections, and respiratory failure. Common airway
disorders such as bronchitis and chronic obstructive pulmonary disease (COPD) also …