cystic fibrosis

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.

Tauroursodeoxycholate (UR 906), also known as ursodoxicoltaurine, is a highly hydrophilic tertiary bile acid that is produced in humans at a low concentration. Tauroursodeoxycholate is the more hydrophilic form of ursodeoxycholic acid, which is the more abundant naturally produced bile acid in humans.Tauroursodeoxycholate is being investigated for use in several conditions such as Primary Biliary Cirrhosis (PBC), insulin resistance, amyloidosis, Cystic Fibrosis, Cholestasis, and Amyotrophic Lateral Sclerosis.

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

VRT-532 (CFpot-532) is an effective modulator of CFTR and is commonly used in studies of cystic fibrosis (CF) caused by CFTR defects. Modification of CFTR by this small molecule modulator can increase arylsulfatase B (ARSB), which is necessary to reduce the accumulation of sulphate glycosaminosaccharide (gag), thus reducing the accumulation of 4-chondroitin sulfate in cystic fibrosis.

Bamocaftor is a CFTR channel (DeltaF508-CFTR mutation) corrector for CF transmembrane conductance regulators designed to restore F508del-CFTR protein function. Combined use of tezacaftor and VX-561 for the treatment of cystic fibrosis patients with F508del MF.

Corrector C4, a corrector commonly used to study cystic fibrosis mutants, works by alleviating the interaction between CFTR transmembrane domain mutants and protein homeostasis.

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].

JNJ-49095397 (RV568) is a selective p38 MAPK-α and p38 MAPK--γ kinase inhibitor with anti-inflammatory activity and potential antiviral activity for chronic obstructive pulmonary disease (COPD), cystic fibrosis, and respiratory syncytial virus infection.

CFTR corrector 12 is a CFTR corrector that rescues all mutant proteins except M760R ABCA3, and can be used to study cystic fibrosis.

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

Ibuprofen (Advil) is a propionic acid derivate and nonsteroidal anti-inflammatory drug (NSAID) with anti-inflammatory, analgesic, and antipyretic effects. Ibuprofen inhibits the activity of cyclo-oxygenase I and II, resulting in a decreased formation of precursors of prostaglandins and thromboxanes.

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

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.

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.

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).

SSR69071 is a potent, orally active, and selective inhibitor of neutrophil elastase, displaying higher affinity for human elastase (Ki = 0.0168 nM) compared to rat (Ki = 3 nM), mouse (Ki = 1.8 nM), and rabbit (Ki = 58 nM) elastases [2]. It reduces myocardial infarct size following ischemia-reperfusion injury [1] and has potential for treating chronic obstructive pulmonary diseases, asthma, emphysema, cystic fibrosis, and various inflammatory diseases.

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.

Talniflumate (Somalgen) is a calcium-activated chloride channel (hCLCA1 mCLCA3) blocker that reduces mucin synthesis and releases in animal models and cell culture. It possesses anti-inflammatory actions via inhibition of Cox (cyclooxygenase) and inhibits Cl- HCO3- exchange activity. It also increases survival in a cystic fibrosis mouse model of the distal intestinal obstructive syndrome.

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.

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].