CFTR

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CFTR Signaling Pathway Map

CFTR Signaling Pathways

CFTR Products

  • All (17)
  • CFTR Inhibitors (6)
  • CFTR Antibodie (1)
  • CFTR Activators (3)
  • CFTR Modulators (6)
  • New CFTR Products
Catalog No. Product Name Information Product Use Citations Product Validations
S0198 PPQ-102 PPQ-102 (CFTR Inhibitor IV) is a potent inhibitor of CFTR. PPQ-102 can completely inhibit CFTR chloride current with IC50 of ~90 nM.
S3272 Steviol (Hydroxydehydrostevic acid) Steviol (Hydroxydehydrostevic acid, Hydroxy Dehydrostevic Acid, NSC 226902), a major metabolite of the sweetening compound stevioside, inhibits CFTR activity, reduces AQP2 expression and promotes AQP2 degradation.
S6003 Ataluren (PTC124) Ataluren (PTC124) selectively induces ribosomal read-through of premature but not normal termination codons, with EC50 of 0.1 μM in HEK293 cells, may provide treatment for genetic disorders caused by nonsense mutations (e.g. CF caused by CFTR nonsense mutation). Phase 3.
Int J Mol Sci, 2022, 23(7)3541
Biomedicines, 2022, 10(4)886
EBioMedicine, 2021, 70:103515
S7139 CFTRinh-172 CFTRinh-172 (CFTR inhibitor 172) is a voltage-independent, selective CFTR inhibitor with Ki of 300 nM, showing no effects on MDR1, ATP-sensitive K+ channels, or a series of other transporters.
Nat Commun, 2022, 13(1):2344
Nat Commun, 2021, 12(1):3332
Cells, 2021, 10(8)2132
S7329 IOWH032 IOWH032 is a synthetic CFTR inhibitor with IC50 of 1.01 μM in CHO-CFTR cell based assays. Phase 2.
S8094 GlyH-101 GlyH-101 is a selective and reversible CFTR inhibitor with Ki of 4.3 μM.
Cell Mol Immunol, 2020, 10.1038/s41423-020-0499-3
A5302 TDP43 Rabbit Recombinant mAb TDP43 Rabbit Recombinant mAb detects endogenous level of total TDP43.
S1144 Ivacaftor (VX-770) Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
Nat Commun, 2022, 13(1):2344
Front Pharmacol, 2022, 13:868863
Front Cell Infect Microbiol, 2022, 12:819554
S6911 Nesolicaftor (PTI-428) Nesolicaftor (PTI-428) is a specific cystic fibrosis transmembrane conductance regulator (CFTR) amplifier that increases CFTR expression.
S8698 GLPG1837 GLPG1837 (ABBV-974) is a novel CFTR potentiator with an EC50 value of 3 nM for F508del, showing enhanced efficacy on CFTR mutants harboring class III mutations compared to Ivacaftor.
iScience, 2022, 25(1):103710
Sci Rep, 2019, 9(1):17640
S1565 Lumacaftor (VX-809) Lumacaftor (VX-809, VRT 826809) acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM in fisher rat thyroid cells. Phase 3.
Mol Syst Biol, 2022, 18(2):e10629
NPJ Genom Med, 2022, 7(1):28
J Cell Sci, 2022, jcs.259002
S6599 KM11060 KM11060 is a small molecule that corrects the processing of cystic fibrosis transmembrane conductance regulator (CFTR) proteins bearing the F508 deletion (F508del) mutation.
S7059 Tezacaftor (VX-661) Tezacaftor (VX-661) is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.
Mol Syst Biol, 2022, 18(2):e10629
FASEB J, 2022, 36(5):e22270
Front Cell Infect Microbiol, 2022, 12:819554
S8535 Galicaftor (ABBV-2222) Galicaftor (ABBV-2222, GLPG2222) is a potent corrector of CFTR for the treatment of Cystic Fibrosis (CF).
S8795 FDL169 FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.
Cell Mol Gastroenterol Hepatol, 2021, S2352-345X(21)00158-2
JCI Insight, 2020, 5(18)139983
S8851 Elexacaftor (VX-445) Elexacaftor (VX-445) is a next-generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector.This is a compound which is not chiral purity.
Cell, 2022, 185(1):158-168.e11
Mol Syst Biol, 2022, 18(2):e10629
iScience, 2022, 25(1):103710
S9972New Icenticaftor (QBW251)

Icenticaftor (QBW251, NVP-QBW251) is an oral potentiator of the cystic fibrosis transmembrane conductance regulator (CFTR) channel with EC50 of 79 nM and 497 nM for F508del CFTR and G551D CFTR in Fisher Rat Thyroid (FRT) cells.

S0198 PPQ-102 PPQ-102 (CFTR Inhibitor IV) is a potent inhibitor of CFTR. PPQ-102 can completely inhibit CFTR chloride current with IC50 of ~90 nM.
S3272 Steviol (Hydroxydehydrostevic acid) Steviol (Hydroxydehydrostevic acid, Hydroxy Dehydrostevic Acid, NSC 226902), a major metabolite of the sweetening compound stevioside, inhibits CFTR activity, reduces AQP2 expression and promotes AQP2 degradation.
S6003 Ataluren (PTC124) Ataluren (PTC124) selectively induces ribosomal read-through of premature but not normal termination codons, with EC50 of 0.1 μM in HEK293 cells, may provide treatment for genetic disorders caused by nonsense mutations (e.g. CF caused by CFTR nonsense mutation). Phase 3.
Int J Mol Sci, 2022, 23(7)3541
Biomedicines, 2022, 10(4)886
EBioMedicine, 2021, 70:103515
S7139 CFTRinh-172 CFTRinh-172 (CFTR inhibitor 172) is a voltage-independent, selective CFTR inhibitor with Ki of 300 nM, showing no effects on MDR1, ATP-sensitive K+ channels, or a series of other transporters.
Nat Commun, 2022, 13(1):2344
Nat Commun, 2021, 12(1):3332
Cells, 2021, 10(8)2132
S7329 IOWH032 IOWH032 is a synthetic CFTR inhibitor with IC50 of 1.01 μM in CHO-CFTR cell based assays. Phase 2.
S8094 GlyH-101 GlyH-101 is a selective and reversible CFTR inhibitor with Ki of 4.3 μM.
Cell Mol Immunol, 2020, 10.1038/s41423-020-0499-3
A5302 TDP43 Rabbit Recombinant mAb TDP43 Rabbit Recombinant mAb detects endogenous level of total TDP43.
S1144 Ivacaftor (VX-770) Ivacaftor (VX-770) is a selective potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.
Nat Commun, 2022, 13(1):2344
Front Pharmacol, 2022, 13:868863
Front Cell Infect Microbiol, 2022, 12:819554
S6911 Nesolicaftor (PTI-428) Nesolicaftor (PTI-428) is a specific cystic fibrosis transmembrane conductance regulator (CFTR) amplifier that increases CFTR expression.
S8698 GLPG1837 GLPG1837 (ABBV-974) is a novel CFTR potentiator with an EC50 value of 3 nM for F508del, showing enhanced efficacy on CFTR mutants harboring class III mutations compared to Ivacaftor.
iScience, 2022, 25(1):103710
Sci Rep, 2019, 9(1):17640
S1565 Lumacaftor (VX-809) Lumacaftor (VX-809, VRT 826809) acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation,EC50 of 0.1 μM in fisher rat thyroid cells. Phase 3.
Mol Syst Biol, 2022, 18(2):e10629
NPJ Genom Med, 2022, 7(1):28
J Cell Sci, 2022, jcs.259002
S6599 KM11060 KM11060 is a small molecule that corrects the processing of cystic fibrosis transmembrane conductance regulator (CFTR) proteins bearing the F508 deletion (F508del) mutation.
S7059 Tezacaftor (VX-661) Tezacaftor (VX-661) is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.
Mol Syst Biol, 2022, 18(2):e10629
FASEB J, 2022, 36(5):e22270
Front Cell Infect Microbiol, 2022, 12:819554
S8535 Galicaftor (ABBV-2222) Galicaftor (ABBV-2222, GLPG2222) is a potent corrector of CFTR for the treatment of Cystic Fibrosis (CF).
S8795 FDL169 FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.
Cell Mol Gastroenterol Hepatol, 2021, S2352-345X(21)00158-2
JCI Insight, 2020, 5(18)139983
S8851 Elexacaftor (VX-445) Elexacaftor (VX-445) is a next-generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector.This is a compound which is not chiral purity.
Cell, 2022, 185(1):158-168.e11
Mol Syst Biol, 2022, 18(2):e10629
iScience, 2022, 25(1):103710
S9972New Icenticaftor (QBW251)

Icenticaftor (QBW251, NVP-QBW251) is an oral potentiator of the cystic fibrosis transmembrane conductance regulator (CFTR) channel with EC50 of 79 nM and 497 nM for F508del CFTR and G551D CFTR in Fisher Rat Thyroid (FRT) cells.

Tags: CFTR inhibitor|CFTR agonist|CFTR activator|CFTR inducer|CFTR antagonist|CFTR signaling pathway|CFTR assay kit