CFTR (Cystic Fibrosis Transmembrane Conductance Regulator)

Sigaling Pathway Map

Research Area

Inhibitory Selectivity

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Notes:
2. For more details, such as half maximal inhibitory concentrations (IC50s) and working concentrations of each inhibitor, please click on the link of the inhibitor of interest.
3. "+" indicates inhibitory effect. Increased inhibition is marked by a higher "+" designation.
4. Orange "√" refers to compounds which do inhibitory effects on the related isoform, but without specific value.

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.

S8094New

GlyH-101

GlyH-101 is a selective and reversible CFTR inhibitor with Ki of 4.3 μM.

S7139

CFTRinh-172

CFTRinh-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.

S1565

VX-809 (Lumacaftor)

VX-809 (Lumacaftor) 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

VX-661

VX-661 is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.

S1144

Ivacaftor (VX-770)

Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.

S1675

Lubiprostone

Lubiprostone is an activator of ClC-2 chloride channels, used in the management of idiopathic chronic constipation.

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.

S8094New

GlyH-101

GlyH-101 is a selective and reversible CFTR inhibitor with Ki of 4.3 μM.

S7139

CFTRinh-172

CFTRinh-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.

Catalog No. Information Product Use Citations Product Validations
S1144

Ivacaftor (VX-770)

Ivacaftor (VX-770) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM in fisher rat thyroid cells, respectively.

2014, 6(246):246ra96

2015, 10(3):363-81

2012, 186(7):694-6

S1675

Lubiprostone

Lubiprostone is an activator of ClC-2 chloride channels, used in the management of idiopathic chronic constipation.

Catalog No. Information Product Use Citations Product Validations
S1565

VX-809 (Lumacaftor)

VX-809 (Lumacaftor) 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.

2013, 19(7):939-45

2014, 6(246):246ra96

2015, 10(3):363-81

S7059

VX-661

VX-661 is a second F508del CFTR corrector and is believed to help CFTR protein reach the cell surface. Phase 2.

2014, 6(246):246ra96