cystic fibrosis transmembrane conductance regulator

Elexacaftor (VX-445) is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector. It promotes the processing and trafficking of CFTR, increases the amount of CFTR on the cell surface, and improves the processing and trafficking of Phe508del CFTR protein.

Tezacaftor (VX661) is a small molecule that can be used as a corrector of the cystic fibrosis transmembrane conductance regulator (CFTR) gene function.

Lumacaftor (VRT 826809) is a CFTR modulator that corrects the folding and trafficking of CFTR protein. It enhances F508del-CFTR protein maturation in FRT cells (EC50: 100 nM).

CFTR corrector 4 is a potent and orally available transmembrane conductance regulator (CFTR) for cystic fibrosis and is a potent (R,R) type active enantiomer. CFTR corrector 4 increases CFTR levels on the cell surface and is a potential compound for the study of cystic fibrosis.

Cavosonstat (N91115) is an orally active S-nitrosoglutathione reductase (GSNOR) inhibitor that promotes cystic fibrosis transmembrane conductance regulator (CFTR) maturation and plasma membrane stabilization. As a CFTR stabilizer, Cavosonstat can be used to study cystic fibrosis.

CFTR corrector 8 is a highly effective modulator of the cystic fibrosis transmembrane conductance regulator (CFTR). This compound is specifically designed for utilization in research related to cystic fibrosis, a genetic disorder primarily affecting the lungs and digestive system [1].

BPO-27 racemate (BPO-27 (racemate)) is an effective CFTR inhibitor with IC50 of 8 nM.

Glibenclamide (Glyburide) is an antidiabetic sulfonylurea derivative with actions similar to those of chlorpropamide.

DNDS is a channel blocker of voltage-dependent cystic fibrosis transmembrane conductance regulator (CFTR).

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

Ataluren (PTC124) is a novel, orally administered drug that targets nonsense mutations. Ataluren is approved for use by the European Medicines Agency to treat Duchenne Muscular Dystrophy in patients aged 5 years and older who are able to walk.

(S)-(+)-Ibuprofen (Dexibuprofen) , is a non-steroidal anti-inflammatory drug (NSAID), inhibiting cyclooxygenase (COX).

CFTR(inh)-172 (CFTR Inhibitor-172) is a voltage-independent, selective CFTR inhibitor.

GlyH-101 is a cell-permeable glycinyl hydrazone compound that blocks CFTR with Ki of 1.4 uM.

CFTR Corrector 9, a cystic fibrosis transmembrane conductance regulator (CFTR) modulator, is employed in the research of cystic fibrosis (CF) and related CFTR-associated disorders [1].

CFTR corrector 2 (FDL169) is a novel and potent CFTR (Cystic fibrosis transmembrane conductance regulator) corrector for treating cystic fibrosis (CF) patients who carry the F508del mutation.

GLPG1837 (ABBV-974) is an effective CFTR potentiator, with EC50s of 3 nM and 339 nM for F508del and G551D CFTR, respectively.

CFTR Corrector 15 (Compound 4172) serves as a corrector for cystic fibrosis transmembrane conductance regulator (CFTR). When used in combination with VX-809, it addresses the folding defects of F508del-CFTR. CFTR Corrector 15 is also applicable in the research of cystic fibrosis.

CFTRcorrector 16 (Compound 39) is a corrector of the cystic fibrosis transmembrane conductance regulator (CFTR), utilized in the research of cystic fibrosis disease.

Tezacaftor-d4 is a deuterated compound of Tezacaftor. Tezacaftor has a CAS number of 1152311-62-0. VX-661 is a small molecule that can be used as a corrector of the cystic fibrosis transmembrane conductance regulator (CFTR) gene function.

CTP-656, a cystic fibrosis transmembrane conductance regulator (CFTR) channel activator, is used potentially for the treatment of cystic fibrosis.

(R)-Elexacaftor, an enantiomer of Elexacaftor, functions as a modulator of the cystic fibrosis transmembrane conductance regulator (CFTR). It exhibits an EC50 of 0.29 µM for the CFTR dF508 mutation [1].

C4 Ceramide is a bioactive sphingolipid and cell-permeable analog of naturally occurring ceramides. [1] [2] [3] It inhibits IL-4 production by 16% in EL4 T cells stimulated with phorbol 12-myristate 13-acetate when used at a concentration of 10 μM. [1] C4 Ceramide is cytotoxic to SK-BR-3 and MCF-7/Adr breast cancer cells (IC50s = 15.9 and 19.9 μM, respectively). [2] C4 Ceramide also increases maturation and stability of cystic fibrosis transmembrane conductance regulator (CFTR) proteins bearing the F508 deletion (F508del) mutation, enhances cAMP-activated chloride secretion, and suppresses secretion of IL-8 in primary epithelial cells isolated from patients with cystic fibrosis.[3]

CFTRcorrector 14 (Compound SVQ26) functions as a Class 3 corrector targeting the cystic fibrosis transmembrane conductance regulator (CFTR), enhancing CFTR activity (improving activity in the presence of Class 1 corrector VX-809 with an EC50 of 3.08 μM). It addresses the misfolding and functional impairments of CFTR proteins caused by mutations. CFTRcorrector 14 is used in cystic fibrosis research.