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-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-103369
    PG01
    Activator 98.04%
    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-B1153R
    Glafenine (Standard)
    Activator
    Glafenine (Standard) is the analytical standard of Glafenine. This product is intended for research and analytical applications. Glafenine (Glafenin) is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF).
    Glafenine (Standard)
  • HY-14832R
    Ataluren (Standard)
    Inhibitor
    Ataluren (Standard) is the analytical standard of Ataluren. This product is intended for research and analytical applications. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.
    Ataluren (Standard)
  • 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-W984122
    CFTR corrector 17
    Modulator 98.05%
    CFTR corrector 17 is an isoxazole compound and CFTR modulator. CFTR corrector 17 can be used for the research of cystic fibrosis.
    CFTR corrector 17
  • HY-109187B
    (R)-Posenacaftor sodium
    Inhibitor 98.96%
    (R)-Posenacaftor (R)-PTI-801) sodium is the R enantiomer of Posenacaftor. Posenacaftor is a cystic fibrosis transmembrane regulator (CFTR) protein modulator that corrects the folding and trafficking of CFTR protein. Posenacaftor is used for the research of cystic fibrosis (CF).
    (R)-Posenacaftor sodium
  • 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-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-13017A
    Ivacaftor benzenesulfonate
    Agonist
    Ivacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
    Ivacaftor benzenesulfonate
  • HY-13017B
    Ivacaftor hydrate
    Agonist
    Ivacaftor hydrate (VX-770 hydrate) is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
    Ivacaftor hydrate
  • HY-145603S
    Vanzacaftor-d4
    Vanzacaftor-d4 (VX-121-d4) is the deuterium labeled Vanzacaftor (HY-145603). Vanzacaftor is an orally active noval corrector of Cystic fibrosis transmembrane conductance regulator (CFTR). Vanzacaftor improves processing and trafficking of CFTR protein as well as increases chloride transport in triple combined with Tezacaftor (HY-15448) and Deutivacaftor. Vanzacaftor-Tezacaftor-Deutivacaftor is safe and well tolerated, improving lung function, respiratory symptoms, and CFTR function with cystic fibrosis, which is promising for research in the field of cystic fibrosis diseases.
    Vanzacaftor-d<sub>4</sub>
  • HY-119179
    UCCF-853
    Modulator 99.93%
    UCCF-853 is a CFTR modulator.
    UCCF-853
  • HY-117187
    Corr4A
    Modulator 98.36%
    Corr4A is a chemical corrector, which can be used for cystic fibrosis. Corr4A interacts directly with the cystic fibrosis transmembrane conductance regulator (CFTR) or affects indirectly its folding process. Corr4A increases the expression of CFTR ΔF508 on the cell surface, thereby improving its transport to the plasma membrane and increasing the stability of the rescued mutant protein.
    Corr4A
  • HY-N6818R
    5,​7,​4'-​Trimethoxyflavone (Standard)
    Activator
    5,​7,​4'-​Trimethoxyflavone (Standard) is the analytical standard of 5,​7,​4'-​Trimethoxyflavone. This product is intended for research and analytical applications. 5,7,4’-Trimethoxyflavone can be isolated from the medicinal plant Kaempferia parviflora (KP). 5,7,4’-Trimethoxyflavone is a CFTR activator and EC50 is 64 μM. 5,7,4’-Trimethoxyflavone induces apoptosis, increases proteolytic activation of caspase-3, and degradation of ADP-ribose polymerase (PARP) protein. 5,7,4’-Trimethoxyflavone has antitumor activity. 5,7,4’-Trimethoxyflavone can be used to prevent skin aging and oxidative stress.
    5,​7,​4'-​Trimethoxyflavone (Standard)
  • HY-133013
    GLPG-3221
    GLPG-3221 is a potent, orally active corrector of CFTR (cystic fibrosis transmembrane conductance regulator), with an EC50 of 105 nM. GLPG-3221 can be uesd for the treatment of cystic fibrosis.
    GLPG-3221
  • HY-109187
    Posenacaftor
    Modulator
    Posenacaftor (PTI-801) is a cystic fibrosis transmembrane regulator (CFTR) protein modulator that corrects the folding and trafficking of CFTR protein. Posenacaftor is used for the research of cystic fibrosis (CF).
    Posenacaftor
  • HY-169000
    CFTR corrector 16
    Modulator
    CFTR corrector 16 (Compound 39) is a corrector for cystic fibrosis transmembrane conductance regulator (CFTR), that can be used in research of cystic fibrosis disease.
    CFTR corrector 16
  • HY-161753
    CFTR potentiator 1
    Activator
    CFTR potentiator 1 (I1421) is a potent CFTR potentiator with an EC50 value of 64 nM. CFTR potentiator 1 allosterically activates a wide range of CF-causing mutants, such as ΔF508 and G551D CFTR.
    CFTR potentiator 1
  • HY-133569
    ARN23765
    Modulator
    ARN23765 is a corrector for F508del-CFTR with an EC50 of 38 pM in human bronchial epithelial cells. ARN23765 improves the maturation and function of F508del-CFTR on the cell membrane, affects the ions transport and secretion, and corrects the pathological mechanism of cystic fibrosis (CF).
    ARN23765
Cat. No. Product Name / Synonyms Application Reactivity