CFTR

Signaling Pathway Map

Research Area

Inhibitory Selectivity

CFTR Products

Catalog No. Information Product Use Citations Product Validations
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.

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.

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.

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.

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.

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.

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.

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.

S8795

FDL169

FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.

S8535

Galicaftor (ABBV-2222)

Galicaftor (ABBV-2222, GLPG2222) is a potent corrector of CFTR for the treatment of Cystic Fibrosis (CF).

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.

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.

Catalog No. Information Product Use Citations Product Validations
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.

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.

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.

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.

Catalog No. Information Product Use Citations Product Validations
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.

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.

Catalog No. Information Product Use Citations Product Validations
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.

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.

S8795

FDL169

FDL169 is a CFTR corrector that is designed to fix and restore the function of the defective CFTR protein.

S8535

Galicaftor (ABBV-2222)

Galicaftor (ABBV-2222, GLPG2222) is a potent corrector of CFTR for the treatment of Cystic Fibrosis (CF).

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.

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.