Signaling Pathway

CFTR

CFTR

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CFTR (Cystic fibrosis transmembrane conductance regulator) is a membrane protein in vertebrates that is encoded by the CFTR gene. CFTR is an ABC transporter-class ion channel that conducts chloride and thiocyanate ions across epithelial cell membranes. Mutations of the CFTR gene affecting chloride ion channel function lead to dysregulation of epithelial fluid transport in the lung, pancreas and other organs, resulting in cystic fibrosis. Complications include thickened mucus in the lungs with frequent respiratory infections, and pancreatic insufficiency giving rise to malnutrition and diabetes. These conditions lead to chronic disability and reduced life expectancy. CFTR functions as a cAMP-activated ATP-gated anion channel, increasing the conductance for certain anions (e.g. Cl–) to flow down their electrochemical gradient. ATP-driven conformational changes in CFTR open and close a gate to allow transmembrane flow of anions down their electrochemical gradient. CFTR is an ion channel that evolved as a 'broken' ABC transporter that leaks when in open conformation.

CFTR Inhibitors & Modulators (11)

Product Name Catalog No. Brief Description
CFTR(inh)-172

HY-16671

CFTR(inh)-172 is an reversibly inhibitor of CFTR short-circuit current in less than 2 minutes in a voltage-independent manner with Ki approximately 300 nM.

GlyH-101

HY-18336

GlyH-101 is a cell-permeable glycinyl hydrazone compound that blocks CFTR with Ki of 1.4 uM.

IOWH-032

HY-18337

iOWH-032 is a novel and potent CFTR inhibitor (IC50=1.01 uM) in T84 and CHO-CFTR cell based assays.

Ivacaftor

HY-13017

Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.

Ivacaftor benzenesulfonate

HY-13017A

Ivacaftor benzenesulfonate (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.

Ivacaftor hydrate

HY-13017B

Ivacaftor hydrate (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.

KM11060

HY-19970

KM11060 is a novel corrector of the F508del-CFTR trafficking defect.

PPQ-102

HY-14179

PPQ-102 is a potent CFTR inhibitor which can completely inhibited CFTR chloride current with IC50 approximately 90 nM.

PTC124

HY-14832

PTC124 (Ataluren) selectively induces ribosomal read-through of premature but not normal termination codons, with EC50 of 0.1 μM, may provide treatment for genetic disorders caused by nonsense mutations (e.

VX-661

HY-15448

VX-661 is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface.

VX-809

HY-13262

VX-809 acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM.