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-P10806
    kCAL01
    		Inhibitor
    kCAL01 is a CAL inhibitor with a Ki value of 2.3 μM. kCAL01 is promising for research of cystic fibrosis (CF).
    kCAL01
  • HY-15206A
    Glibenclamide potassium
    		Inhibitor
    Glibenclamide (Glyburide) potassium is a potassium salt of Glibenclamide (HY-15206). Glibenclamid potassium exists in anhydrous and hydrate forms, with higher solubility compared to pure Glibenclamide.
    Glibenclamide potassium
  • HY-126394B
    Bamocaftor potassium
    		Modulator
    Bamocaftor potassium is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore F508del-CFTR protein function. Bamocaftor potassium can be used combine with Tezacaftor (HY-15448) and Ivacaftor (HY-13017) in cystic fibrosis research.
    Bamocaftor potassium
  • HY-145126
    CP-628006
    		Activator
    CP-628006, a small molecule CFTR potentiator, restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR.
    CP-628006
  • HY-109027R
    Cavosonstat (Standard)
    		Modulator
    Cavosonstat (Standard) is the analytical standard of Cavosonstat (HY-109027). This product is intended for research and analytical applications. 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 (Standard)
  • HY-111772R
    Elexacaftor (Standard)
    		Modulator
    Elexacaftor (Standard) is the analytical standard of Elexacaftor. This product is intended for research and analytical applications. Elexacaftor (VX-445, Compound 1) is a modulator of cystic fibrosis transmembrane conductance regulator (CFTR). Elexacaftor (VX-445, Compound 1) facilitates the processing and trafficking of CFTR to increase the amount of CFTR at the cell surface.
    Elexacaftor (Standard)
  • HY-106203R
    Crinecerfont (Standard)
    		Antagonist
    Crinecerfont (Standard) is the analytical standard of Crinecerfont (HY-106203). This product is intended for research and analytical applications. Crinecerfont (SSR-125543) is an orally effective corticotropin-releasing factor receptor type-1 (CRF1 receptor) antagonist. Crinecerfont blocks CRF1 receptor signaling to reduce adrenocorticotropic hormone secretion. Crinecerfont improves hypothalamic-pituitary-adrenal axis negative feedback sensitivity in chronically stressed mice. Crinecerfont can be used for the research of chronic stress conditions and classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Crinecerfont is a click chemistry reagent, it contains an Alkyne group and can undergo copper-catalyzed azide-alkyne cycloaddition (CuAAc) with molecules containing Azide groups.
    Crinecerfont (Standard)
  • HY-15206S2
    Glibenclamide-13C6
    		Inhibitor
    Glibenclamide-13C6 (Glyburide-13C6) is 13C 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.
    Glibenclamide-<sup>13</sup>C<sub>6</sub>
  • HY-13262R
    Lumacaftor (Standard)
    		Modulator
    Lumacaftor (Standard) is the analytical standard of Lumacaftor. This product is intended for research and analytical applications. Lumacaftor (VX-809; VRT 826809) is a CFTR modulator that corrects the folding and trafficking of CFTR protein.
    Lumacaftor (Standard)
  • HY-B0679R
    Lubiprostone (Standard)
    		Activator
    Lubiprostone (Standard) is the analytical standard of Lubiprostone. This product is intended for research and analytical applications. Lubiprostone (SPI-0211) increases intestinal fluid secretion through generation of CIC-2/CFTR and activation of cAMP signaling pathway. Lubiprostone inhibits myeloperoxidase (MPO) activity, downregulates Indomethacin (HY-14397)-induced iNOS and TNFα expression. Lubiprostone can be used for chronic constipation research.
    Lubiprostone (Standard)
  • HY-169536
    CFTR activator 2
    		Activator 99.54%
    CFTR activator 2 (WAY-326769) is an activator of mutant cystic fibrosis transmembrane conductance regulator (mutant-CFTR).
    CFTR activator 2
  • HY-N2057R
    Steviol (Standard)
    		Inhibitor
    Steviol (Standard) is the analytical standard of Steviol (HY-N2057). This product is intended for research and analytical applications. Steviol is the main metabolite of steviol glycosides and an inhibitor of AQP2/CFTR. Steviol slows down the growth of renal cysts by inhibiting the activity of CFTR, reducing the expression of AQP2, and promoting the degradation of AQP2 and CFTR. Steviol can be used in the research of polycystic kidney disease.
    Steviol (Standard)
  • HY-134385
    6-PhEt-dATP
    6-PhEt-dATP is an ATP analog that specifically enhances the function of certain CFTR mutants and is useful for studying cystic fibrosis.
    6-PhEt-dATP
  • HY-13017S4
    Ivacaftor-13C6
    		Agonist
    Ivacaftor-13C6 (VX-770-13C6) is 13C 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-<sup>13</sup>C<sub>6</sub>
  • HY-14832S1
    Ataluren-13C6
    		Inhibitor
    Ataluren-13C6 (PTC124-13C6) is 13C labeled Ataluren. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.
    Ataluren-<sup>13</sup>C<sub>6</sub>
  • HY-P10226
    PGD97
    		Activator
    PGD97 is a selective cyclic peptide inhibitor against CAL/CFTR interactions, with a KD value of 6 nM towards the CAL PDZ domain for its desulfide cyclized form. PGD97 (desulfide cyclized form) has selectivity ≥ 130-fold compared to NHERF1/2 PDZ domains. PGD97 is capable of stabilizing F508del-CFTR at the cell membrane and improving CFTR function required for proper fluid homeostasis in tne lung. PGD97 can be used for the research of cystic fibrosis.
    PGD97
  • HY-B0679S
    Lubiprostone-d7
    		Activator
    Lubiprostone-d7 (RU-0211-d7) is the deuterium labeled Lubiprostone. Lubiprostone (SPI-0211) increases intestinal fluid secretion through generation of CIC-2/CFTR and activation of cAMP signaling pathway. Lubiprostone inhibits myeloperoxidase (MPO) activity, downregulates Indomethacin (HY-14397)-induced iNOS and TNFα expression. Lubiprostone can be used for chronic constipation research.
    Lubiprostone-d<sub>7</sub>
  • HY-B1153AR
    Glafenine hydrochloride (Standard)
    		Activator
    Glafenine (hydrochloride) (Standard) is the analytical standard of Glafenine (hydrochloride). This product is intended for research and analytical applications. Glafenine (Glafenin) hydrochloride is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine hydrochloride exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine hydrochloride can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine hydrochloride can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF).
    Glafenine hydrochloride (Standard)
  • HY-153133
    CFTR corrector 11
    		Modulator 99.80%
    CFTR corrector 11 (compound 133) is a CFTR corrector. CFTR corrector 11 can be used for the study of cystic fibrosis.
    CFTR corrector 11
  • HY-15448S1
    Tezacaftor-d6
    		Modulator
    Tezacaftor-d6 (VX-661-d6) is deuterium labeled Tezacaftor. Tezacaftor (VX-661) is a F508del CFTR corrector. It helps CFTR protein reach the cell surface. However, Ivacaftor (VX-770, HY-13017), a CFTR potentiator, helps to prolong the opening time of cell surface CFTR protein channels. Tezacaftor combining with Ivacaftor, shows potent efficacy against cystic fibrosis and diseases with homozygous for the CFTR Phe508del mutation. Moreover, Elexacaftor (VX-445, HY-111772) is also a CFTR corrector. Elexacaftor-Tezacaftor-Ivacaftor aims at with cystic fibrosis (CF) with at least one Phe508del mutation, often avoids the indication for lung transplantation.
    Tezacaftor-d<sub>6</sub>
Cat. No. Product Name / Synonyms Application Reactivity