PPQ-102 is a potent and voltage-independent CFTR inhibitor, which completely inhibit CFTR chloride current with IC50 approximately 90 nM.

CFTR (inh)-172 is an reversibly inhibitor of CFTR short-circuit current in less than 2 minutes in a voltage-independent manner with Ki ~300 nM.

GLPG-1837 is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator1, shows enhanced efficacy on CFTR mutants harboring Class III mutations compared to Ivacaftor.

Ivacaftor is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively.

IOWH-032 is a potent CFTR inhibitor (IC50=1.01 μM) in T84 and CHO-CFTR cell based assays.

GlyH-101, a glycinyl hydrazone compound, is a reversible and voltage-dependent CFTR chloride channel blocker with Ki of 4.3 μM.

FDL169 is CFTR inhibitor. It is an experimental therapy for cystic fibrosis (CF). Phase II.

KM11060 corrects the F508del-CFTR trafficking defect, also shows inhibition on PDE5 activity.

Elexacaftor, also known as VX-445, is a next-generation cystic fibrosis transmembrane conductance regulator (CFTR) corrector.

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

VX-809 acts to correct CFTR mutations common in cystic fibrosis by increasing mutant CFTR (F508del-CFTR) maturation with an EC50 of 0.1 μM.

PTC-124 is nonsense allele inhibitor, used to treat Duchenne muscular dystrophy.

Lubiprostone is an ClC-2 chloride channel activator used in the treatment of idiopathic chronic constipation.