1. Membrane Transporter/Ion Channel
  2. CFTR

CFTR

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 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 Related Products (13):

Cat. No. Product Name Effect Purity
  • HY-13262
    VX-809 Modulator 99.12%
    VX-809 is a CFTR corrector, which improves F508del-CFTR maturation with EC50 of 0.1±0.1 μM.
  • HY-13017
    Ivacaftor Agonist 98.36%
    Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.
  • HY-14832
    PTC124 Inhibitor 99.88%
    PTC124 targets nonsense mutations, which insert a termination codon in the middle of the CFTR gene.
  • HY-16671
    CFTR(inh)-172 Inhibitor 98.87%
    CFTR(inh)-172 is a potent and selective blocker of the CFTR chloride channel; reversibly inhibited CFTR short-circuit current in less than 2 minutes with a Ki of 300 nM.
  • HY-18336
    GlyH-101 Inhibitor 99.35%
    GlyH-101 is a cell-permeable glycinyl hydrazone compound that blocks CFTR with Ki of 1.4 uM.
  • HY-15448
    VX-661 Modulator 99.56%
    VX-661 is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface.
  • HY-19778A
    BPO-27 racemate Inhibitor 98.67%
    BPO-27 racemate is a potent CFTR inhibitor with an IC50 of 8 nM.
  • HY-19778
    (R)-BPO-27 Inhibitor
    (R)-BPO-27 is a potent CFTR inhibitor with an IC50 of 4 nM.
  • HY-18337
    IOWH-032 Inhibitor
    iOWH-032 is a novel and potent CFTR inhibitor (IC50=1.01 uM) in T84 and CHO-CFTR cell based assays.
  • HY-14179
    PPQ-102 Inhibitor 98.19%
    PPQ-102 is a potent CFTR inhibitor which can completely inhibited CFTR chloride current with IC 50approximately 90 nM.
  • HY-19970
    KM11060 Activator
    KM11060 is a novel corrector of the F508del-CFTR trafficking defect.
  • HY-13017A
    Ivacaftor benzenesulfonate Agonist
    Ivacaftor benzenesulfonate (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.
  • HY-13017B
    Ivacaftor hydrate Agonist
    Ivacaftor hydrate (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.