The most common mutation causing cystic fibrosis (CF), F508del, impairs conformational
maturation of CF transmembrane conductance regulator (CFTR), thereby reducing its …

Cystic fibrosis results from mutations in the cystic fibrosis transmembrane conductance
regulator (CFTR) chloride channel, leading to defective apical chloride transport. Patients also …

The deletion of Phe-508 (ΔPhe508) constitutes the most prevalent of a number of mutations
in the cystic fibrosis transmembrane conductance regulator (CFTR) that cause cystic fibrosis (…

The cystic fibrosis transmembrane conductance regulator (CFTR) attenuates sphingosine-1-phosphate
(S1P) signaling in resistance arteries and has emerged as a prominent regulator …

High-throughput screens for small molecules that are effective in “correcting” the functional
expression of F508del-CFTR have yielded several promising hits. Two such compounds are …

CFTR is a member of the ABC (ATP binding cassette) superfamily of transporters. It is a
multidomain membrane protein, which utilizes ATP to regulate the flux of its substrate through the …

Next we investigated whether addition of 5 mM L-arginine affects the activation of a2-adrenoceptors.
It was shown that in the presence of a2-adrenoceptor antagonists-yohimbine (10 …

The major cystic fibrosis causing mutation, F508del‐CFTR (where CFTR is cystic fibrosis
transmembrane conductance regulator), impairs biosynthetic maturation of the CFTR protein, …

These are exciting times with the appearance of small molecule compounds in clinical trials
which target the basic defects caused by mutation in the CFTR gene. This progress was …

The resistance to coronary blood flow in various parts of the myocardium was studied with
the tracer microspheres technique before and immediately after an acute coronary occlusion …