1. Signaling Pathways
  2. Membrane Transporter/Ion Channel
  3. CFTR

CFTR

Cystic fibrosis transmembrane conductance regulator

CFTR (Cystic fibrosis transmembrane conductance regulator), mutations of which cause cystic fibrosis, belongs to the ATP-binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. CFTR is composed of two homologous halves, each comprising a transmembrane (TMD) and a nucleotide binding domain (NBD). CFTR activity is regulated by phosphorylation of its cytosolic regulatory (R) domain, and ATP binding and hydrolysis at two NBDs.

CFTR is expressed in many cell types throughout the body, but in the airways it is found mainly in secretory serous cells of the submucosal glands. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. CFTR function is normally tightly controlled as dysregulation can lead to life-threatening diseases such as secretory diarrhoea and cystic fibrosis.

Cat. No. Product Name Effect Purity Chemical Structure
  • HY-119936
    GLPG2451
    Activator 99.68%
    GLPG2451 is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, which effectively potentiates low temperature rescued F508del CFTR with an EC50 of 11.1 nM.
    GLPG2451
  • HY-13017S3
    Ivacaftor-d4
    Agonist
    Ivacaftor-d4 (VX-770-d4) is the deuterium labeled-Ivacaftor (HY-13017). Ivacaftor is a potent and orally active CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively.
    Ivacaftor-d4
  • HY-155742
    CFTR corrector 12
    Modulator 99.85%
    CFTR corrector 12 (compound 17C) is a bithiazole derivative, serving as CFTR corrector. CFTR corrector 12 has the ability to correct some folding defective mutants of the channel responsible for the control of chloride transport across the plasma membrane. CFTR corrector 12 recovers the α-sarcoglycan (α-SG) content in mutant cells.
    CFTR corrector 12
  • HY-P1106A
    K41498 TFA
    Antagonist 99.40%
    K41498 TFA is a potent and highly selective CRF2 receptor antagonist with Ki values of 0.66 nM, 0.62 nM and 425 nM for human CRF, CRF and CRF1 receptors respectively. K41498 TFA is an analogues of antisauvagine-30 (aSvg-30), inhibits sauvagine-stimulated cAMP accumulation in hCRF- and hCRF-expressing cells. K41498 TFA can be used for hypotension study.
    K41498 TFA
  • HY-164000
    CFTR corrector 15
    Modulator ≥98.0%
    CFTR corrector 15 (Compound 4172) is a corrector for cystic fibrosis transmembrane conductance regulator (CFTR), that repairs the F508del-CFTR folding defect when used with VX-809 (HY-13262). CFTR corrector 15 can be used in research of cystic fibrosis disease.
    CFTR corrector 15
  • HY-158991
    I1421
    Agonist 98.06%
    I1421 is an activator of the cystic fibrosis transmembrane conductance regulator (CFTR) with an EC50 of 64 nM for WT CFTR currents. I1421 also allosterically activates multiple mutants causing cystic fibrosis (CF) with good in vivo potency, with an oral bioavailability of 60% in mice corresponding to a half-life of 75 min. I1421 synergizes with Elexacaftor (HY-111772) to enhance CFTR currents.
    I1421
  • HY-136939
    CFTR corrector 6
    Activator 98.15%
    CFTR corrector 6 is a potent potentiator of Cystic Fibrosis Transmembrane conductance Regulator (CFTR). CFTR corrector 6 has the potential for cystic fibrosis (CF) and other CFTR associated disorders research.
    CFTR corrector 6
  • HY-152264
    CFTR activator 1
    Activator 99.60%
    CFTR activator 1 is a potent and selective CFTR activator, with an EC50 of 23 nM. CFTR activator 1 can be used to ameliorate dry eye disease.
    CFTR activator 1
  • HY-143344
    NJH-2-056
    Modulator 98.23%
    NJH-2-056 is a deubiquitinase-targeting chimera (DUBTAC) linking the OTUB1 recruiter EN523 to the CFTR chaperone lumacaftor. NJH-2-056 can be used for cystic fibrosis research.
    NJH-2-056
  • HY-103369
    PG01
    Activator ≥98.0%
    PG01 is a potent CFTR Cl- channel potentiator. PG01 can correct gating defects of CFTR mutants, is effective on b>E193K, G970R and G551D (CFTR mutants) with Kd values of 0.22 μM, 0.45 μM and 1.94 μM, respectively. PG01 is also effective on ΔF508 (Ka of 0.3 μM). PG01 increases ΔF508-CFTR Cl- current after adding Forskolin.
    PG01
  • HY-111772A
    (R)-Elexacaftor
    Modulator 99.44%
    (R)-Elexacaftor is an enantiomer of Elexacaftor (HY-111772). (R)-Elexacaftor is the Compound 37 from patent WO2018107100A1. (R)-Elexacaftor is a modulator of cystic fibrosis transmembrane conductance regulator (CFTR), the EC50 for CFTR dF508 is 0.29 uM.
    (R)-Elexacaftor
  • HY-135279
    CFTR corrector 4
    Modulator 99.60%
    CFTR corrector 4 (Compound 13), an active (R,R)-form enantiomer, is a highly potent and orally active cystic fibrosis transmembrane conductance regulator (CFTR) corrector. CFTR corrector 4 can increase CFTR levels at the cell surface and have the potential for treatment of cystic fibrosis.
    CFTR corrector 4
  • HY-125381
    CFTR corrector 2
    Modulator 98.92%
    CFTR corrector 2 is a cystic fibrosis transmembrane conductance corrector (CFTR), extracted from patent US20140274933.
    CFTR corrector 2
  • HY-13017S1
    Ivacaftor-d19
    Agonist
    Ivacaftor-d9 is a potent CFTR modulator and exhibits an EC50 value of 255 nM for CFTR potentiation in G551D/F508del HBE Cells. Ivacaftor-D9 acts as an orally active and improved deuterated Ivacaftor analog for cystic fibrosis research.
    Ivacaftor-d<sub>19</sub>
  • HY-13017S2
    Ivacaftor-d18
    Ivacaftor-d18 is the deuterium labeled Ivacaftor[1]. Ivacaftor (VX-770) is a potent and orally bioavailable CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively[2].
    Ivacaftor-d<sub>18</sub>
  • HY-N5101
    Kobusin
    Activator 99.93%
    Kobusin is a bisepoxylignan isolated from the Pnonobio biondii Pamp. Kobusin is an activator of CFTR and CaCCgie chloride channels and a inhibitor of ANO1/CaCC (calcium-activated chloride channel) channel.
    Kobusin
  • HY-15448S
    Tezacaftor-d4
    Modulator
    Tezacaftor-d4 (VX-661-d4) is the deuterium-labeled Tezacaftor (HY-15448), a F508del CFTR corrector. Tezacaftor helps CFTR protein reach the cell surface.
    Tezacaftor-d<sub>4</sub>
  • HY-153911
    H2-Gamendazole
    98.20%
    H2-Gamendazole is a derivative of Lonidamine that reduces cyst formation in polycystic kidney disease and is used in autosomal dominant polycystic kidney disease research.
    H2-Gamendazole
  • HY-147249
    CFTR corrector 8
    Modulator
    CFTR corrector 8 is a potent CFTR modulator. CFTR can be used in the research of cystic fibrosis.
    CFTR corrector 8
  • HY-13017A
    Ivacaftor benzenesulfonate
    Agonist
    Ivacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
    Ivacaftor benzenesulfonate
Cat. No. Product Name / Synonyms Application Reactivity