… past 20 years about the cystic fibrosis transmembrane conductance regulator (CFTR) and to
… of new therapies for cystic fibrosis (CF). The basic ideas are to consider what the CFTR is, …

… CFTR functions as a channel with specificity for anions, in particular chloride and bicarbonate.
In this review we look at what is known about CFTR structure and function … : Cystic Fibrosis…

… cystic fibrosis transmembrane conductance regulator (CFTR) … and acquired CFTR dysfunction
in patients with cystic fibrosis (… mutant CFTR function. a) In normal airway epithelial cells, …

… Here, we review selectively progress to understand how CF mutations disrupt CFTR function.
We explore CFTR structure and function to explain the molecular mechanisms of CFTR …

… Cystic fibrosis (CF) transmembrane conductance … CFTR (zCFTR). Five areas will be
covered: roles of the R domain in CFTR function, asymmetrical pore and gate in CFTR, CFTR’s …

… The cystic fibrosis transmembrane conductance regulator (CFTR) is a unique member of the
… Dysfunction of CFTR causes the genetic disease cystic fibrosis. The CFTR is composed of …

… targets the underlying defect and improves CFTR function in a subgroup of patients in a …
adopted to improve CFTR function: potentiators, which recover the function of CFTR at the apical …

… CFTR function in about 90 percent of patients with cystic fibrosis who had stop mutations in
CFTR… After gentamicin treatment, the electrophysiological abnormalities caused by the CFTR …

… CFTR function and improve CF symptoms in vivo. Here we report the therapeutic effect of …
on Cftr F508del mice as well as the capacity of cysteamine to improve CFTR function of ex …

… This short-term phase I/II study demonstrates proof of principle that modulation of ΔF508
CFTR biosynthesis and trafficking is a viable therapeutic approach for cystic fibrosis. …