Cystic fibrosis transmembrane conductance regulator (CFTR),a cAMP-activated Cl channel, is expressed in epithelial cells in the airways, sweat duct, testis, pancreas,intestine and other fluid-transporting tissues. CFTR is related to cystic fibrosis which is a hereditary lethal disease.CFTR is a large transmembrane glycoprotein containing two six-helix membrane-spanning domains, each followed by a nucleotide-binding domain (NBD), with a regulatory domain linking the first NBDand the second membrane-spanning domain Cytosolic cAMP can activate CFTR by promoting CFTR phosphorylation and blocking CFTR dephosphorylation at the same time.
|Cat.No.||Product Name||Information||Publication||Customer Review|
|S7059||VX-661||VX-661 is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface.|
|S1565||VX-809||VX-809 is a CFTR modulator with EC50 of 0.1 μM.||(2)||(1)|
|S6003||Ataluren (PTC124)||PTC124 (Ataluren) is a potent nonsense mutation inhibitor with EC50 of ~0.1 μM.||(5)||(1)|