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

CFTR

Cystic fibrosis transmembrane conductance regulator

CFTR

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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-152264
    CFTR activator 1
    		Activator 99.94%
    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-19778A
    BPO-27 racemate
    		Inhibitor 98.50%
    BPO-27 racemate is a potent CFTR inhibitor with an IC50 of 8 nM.
    BPO-27 racemate
  • HY-126394
    Bamocaftor
    		Modulator 99.09%
    Bamocaftor (VX-659) is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore F508del-CFTR protein function. Bamocaftor can be used combine with Tezacaftor (HY-15448) and Ivacaftor (HY-13017) in cystic fibrosis research.
    Bamocaftor
  • HY-112267
    Olacaftor
    		Modulator 99.60%
    Olacaftor (VX-440) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator extracted from patent US9782408.
    Olacaftor
  • HY-119229
    VRT-325
    		Modulator 99.58%
    VRT-325 is a CFTR modulator. VRT-325 inhibits disulfide cross-linking between cysteines in transmembrane segments 6 and 7 of CFTR and P-gp. VRT-325 promotes maturation of CFTR and P-gp processing mutants, rescues ΔF508-CFTR folding at the endoplasmic reticulum. VRT-325 binds ΔF508-CFTR nucleotide-binding domain 1, and increases mature ΔF508-CFTR cell surface expression and chloride conductance. VRT-325 can be used for the research of cystic fibrosis[1][3].
    VRT-325
  • 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-111772A
    (R)-Elexacaftor
    		Modulator 99.44%
    (R)-Elexacaftor ((R)-VX-445) is the enantiomer of Elexacaftor (HY-111772). Elexacaftor is an orally active CFTR modulator that targets nucleotide-binding domain 1. Elexacaftor stabilizes misfolded F508del-CFTR protein, enhances its trafficking to the plasma membrane, and significantly improves metabolic stability, thermal stability and ion conductivity. Elexacaftor not only restores chloride transport function in nasal epithelial cells and rescues multiple CFTR mutation subtypes, but also exerts multiplicative synergistic effects with Ivacaftor (HY-13017), and is often used in a triple combination therapy with Tezacaftor (HY-15448). Elexacaftor is widely used in basic and clinical translational research on cystic fibrosis.
    (R)-Elexacaftor
  • HY-19970
    KM11060
    		Activator 98.71%
    KM11060 is a corrector of the F508 deletion (F508del)-cystic fibrosis transmembrane conductance regulator (CFTR) trafficking defect. KM11060 can be used for the research of F508del-CFTR processing defect and development of cystic fibrosis research.
    KM11060
  • 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-109027
    Cavosonstat
    		Modulator 99.36%
    Cavosonstat (N91115) is an orally active S-nitrosoglutathione reductase (GSNOR) inhibitor. Cavosonstat is a CFTR stabilizer, and can be used for cystic fibrosis research.
    Cavosonstat
  • HY-143344
    NJH-2-056
    		Modulator 98.31%
    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-15448S
    Tezacaftor-d4
    		Modulator 98.95%
    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-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-d<sub>4</sub>
  • HY-13017S2
    Ivacaftor-d18
    Ivacaftor-d18 is the deuterium labeled Ivacaftor. 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.
    Ivacaftor-d<sub>18</sub>
  • HY-15206S
    Glyburide-d11
    		Inhibitor 99.39%
    Glyburide-d11 is the deuterium labeled Glibenclamide. Glibenclamide (Glyburide) is an orally active ATP-sensitive K+ channel (KATP) inhibitor and can be used for the research of diabetes and obesity. Glibenclamide inhibits P-glycoprotein. Glibenclamide directly binds and blocks the SUR1 subunits of KATP and inhibits the cystic fibrosis transmembrane conductance regulator protein (CFTR). Glibenclamide interferes with mitochondrial bioenergetics by inducing changes on membrane ion permeability. Glibenclamide can induce autophagy.
    Glyburide-d<sub>11</sub>
  • HY-135279
    CFTR corrector 4
    		Modulator 99.72%
    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-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-15448A
    (Rac)-Tezacaftor
    		Modulator
    (Rac)-Tezacaftor ((Rac)-VX-661) is a racemate of Tezacaftor (HY-15448). Tezacaftor is a F508del CFTR corrector. (Rac)-Tezacaftor can be used for the research of cystic fibrosis.
    (Rac)-Tezacaftor
  • HY-164000
    CFTR corrector 15
    		Modulator 99.67%
    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-13017S1
    Ivacaftor-d19
    		Agonist 98.10%
    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>
Cat. No. Product Name / Synonyms Application Reactivity