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Cystic fibrosis

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141

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5

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8

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Targets Recommended:
Cat. No. Product Name Target Research Areas Chemical Structure
  • HY-15206
    Glibenclamide
    25+ Cited Publications

    Glyburide

    Potassium Channel Mitochondrial Metabolism Autophagy CFTR P-glycoprotein Metabolic Disease
    Glibenclamide (Glyburide) is an orally active ATP-sensitive K + channel (KATP) inhibitor and can be used for the research of diabetes and obesity . Glibenclamide inhibits P-glycoprotein. Glibenclamide directly binds and blocks the SUR1 subunits of KATP and inhibits the cystic fibrosis transmembrane conductance regulator protein (CFTR) . Glibenclamide interferes with mitochondrial bioenergetics by inducing changes on membrane ion permeability . Glibenclamide can induce autophagy .
    Glibenclamide
  • HY-111772
    Elexacaftor
    Maximum Cited Publications
    48 Publications Verification

    VX-445

    CFTR Autophagy Inflammation/Immunology
    Elexacaftor (VX-445, Compound 1) is a modulator of cystic fibrosis transmembrane conductance regulator (CFTR). Elexacaftor (VX-445, Compound 1) facilitates the processing and trafficking of CFTR to increase the amount of CFTR at the cell surface .
    Elexacaftor
  • HY-15448
    Tezacaftor
    20+ Cited Publications

    VX-661

    CFTR Cancer
    Tezacaftor (VX-661) is a F508del CFTR corrector. It helps CFTR protein reach the cell surface. However, Ivacaftor (VX-770, HY-13017), a CFTR potentiator, helps to prolong the opening time of cell surface CFTR protein channels. Tezacaftor combining with Ivacaftor, shows potent efficacy against cystic fibrosis and diseases with homozygous for the CFTR Phe508del mutation. Moreover, Elexacaftor (VX-445, HY-111772) is also a CFTR corrector. Elexacaftor-Tezacaftor-Ivacaftor aims at with cystic fibrosis (CF) with at least one Phe508del mutation, often avoids the indication for lung transplantation .
    Tezacaftor
  • HY-145603
    Vanzacaftor
    4 Publications Verification

    VX-121

    CFTR Chloride Channel Neurological Disease
    Vanzacaftor (VX-121) is an orally active noval corrector of Cystic fibrosis transmembrane conductance regulator (CFTR). Vanzacaftor improves processing and trafficking of CFTR protein as well as increases chloride transport in triple combined with Tezacaftor (HY-15448) and Deutivacaftor. Vanzacaftor-Tezacaftor-Deutivacaftor is safe and well tolerated, improving lung function, respiratory symptoms, and CFTR function with cystic fibrosis, which is promising for research in the field of cystic fibrosis diseases .
    Vanzacaftor
  • HY-114231B
    Exaluren disulfate
    2 Publications Verification

    ELX-02 disulfate; NB-124 disulfate

    DNA/RNA Synthesis Inflammation/Immunology
    Exaluren (ELX-02; NB-124) disulfate is an synthetic eukaryotic ribosome-selective glycoside that induces read-through of nonsense mutations, resulting in normally localized full-length functional proteins. Exaluren disulfate is used for the research of cystic fibrosis caused by nonsense mutations .
    Exaluren disulfate
  • HY-13017S

    VX-770-d9

    CFTR Cancer
    Ivacaftor-d9 is a potent CFTR modulator and exhibits an EC50 value of 255 nM for CFTR potentiation in G551D/F508del HBE Cells. Ivacaftor-D9 acts as an orally active and improved deuterated Ivacaftor analog for cystic fibrosis research .
    Ivacaftor-d9
  • HY-150090
    SRI-41315
    1 Publications Verification

    CFTR Inflammation/Immunology
    SRI-41315 induces a prolonged pause at stop codons and suppresses PTCs (premature termination codons) associated with cystic fibrosis in immortalized and primary human bronchial epithelial cells, restoring CFTR (cystic fibrosis transmembrane conductance regulator) expression and function. SRI-41315 suppresses PTCs by reducing the abundance of the termination factor eRF1. SRI-41315 also potentiates aminoglycoside-mediated readthrough, leading to synergistic increases in CFTR activity .
    SRI-41315
  • HY-N2388

    MMP PPAR Bacterial Cardiovascular Disease Neurological Disease Inflammation/Immunology Cancer
    Auraptene is an orally active geranyloxycoumarin that can be isolated from plants in the Brassicaceae family, antibacterial, anti-pathogen, antioxidant, anti-tumor, and neuroprotective effects. Auraptene plays an important role in the treatment of various chronic diseases such as hypertension and cystic fibrosis .
    Auraptene
  • HY-108858
    Dornase alfa
    1 Publications Verification

    rhDNase

    DNA/RNA Synthesis Endonuclease Inflammation/Immunology
    Dornase alfa (rhDNase) is a recombinant human deoxyribonuclease I (rhDNase) that can specifically degrade extracellular DNA. Dornase alfa catalyzes the cleavage of DNA released by neutrophils in respiratory mucus, reduces sputum viscosity, thereby improving mucus clearance efficiency, reducing airway obstruction and alleviating inflammatory responses. Dornase alfa can be used to improve lung function (such as FEV_1) in cystic fibrosis (CF), reduce the risk of acute pulmonary exacerbations, and has good in vivo tolerability. Dornase alfa acts locally on the respiratory tract through aerosol inhalation, specifically improving the high viscosity of mucus caused by DNA accumulation and related respiratory symptoms .
    Dornase alfa
  • HY-111111
    Galicaftor
    1 Publications Verification

    ABBV-2222; GLPG-2222

    CFTR Inflammation/Immunology
    Galicaftor (ABBV-2222; GLPG-2222) is a potent and orally active cystic fibrosis transmembrane conductance regulator (CFTR) corrector. Galicaftor can be used for cystic fibrosis research .
    Galicaftor
  • HY-145603A
    (R)-Vanzacaftor
    1 Publications Verification

    (R)-VX-121

    CFTR Inflammation/Immunology
    (R)-Vanzacaftor ((R)-VX-121) is a Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator with max activity of 30-60% and EC50 <1 μM in enteroid cells. (R)-Vanzacaftor can be used for the research of cystic fibrosis .
    (R)-Vanzacaftor
  • HY-126394
    Bamocaftor
    1 Publications Verification

    VX-659

    CFTR Endocrinology
    Bamocaftor (VX-659) is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore F508del-CFTR protein function. Bamocaftor can be used combine with Tezacaftor (HY-15448) and Ivacaftor (HY-13017) in cystic fibrosis research .
    Bamocaftor
  • HY-106302

    Moli1901; Lancovutide

    Bacterial Infection Inflammation/Immunology
    Duramycin (Moli1901) is a lantibiotic derived from Streptomyces cinnamoneuma. Duramycin also is a antimicrobial peptide. Duramycin can be used for the research of cystic fibrosis (CF) .
    Duramycin
  • HY-103370

    BA 7602-06

    Chloride Channel Inflammation/Immunology
    Talniflumate (BA 7602-06) is the proagent of Niflumic acid (HY-B0493), exerting its activity in the body through conversion to niflumic acid by esterase . Talniflumate is an orally active Ca 2+-activated Cl - channel (CaCC) blocker. Talniflumate can be used as an analgesic and anti-inflammatory agent in cystic fibrosis mouse model of distal intestinal obstructive syndrome .
    Talniflumate
  • HY-114231
    Exaluren
    2 Publications Verification

    ELX-02; NB-124

    Drug Derivative Inflammation/Immunology
    Exaluren (ELX-02; NB-124) is an synthetic eukaryotic ribosome-selective glycoside that induces read-through of nonsense mutations, resulting in normally localized full-length functional proteins. Exaluren is used for the research of cystic fibrosis caused by nonsense mutations .
    Exaluren
  • HY-111772A

    (R)-VX-445

    Drug Isomer CFTR Inflammation/Immunology
    (R)-Elexacaftor ((R)-VX-445) is the enantiomer of Elexacaftor (HY-111772). Elexacaftor is an orally active CFTR modulator that targets nucleotide-binding domain 1. Elexacaftor stabilizes misfolded F508del-CFTR protein, enhances its trafficking to the plasma membrane, and significantly improves metabolic stability, thermal stability and ion conductivity. Elexacaftor not only restores chloride transport function in nasal epithelial cells and rescues multiple CFTR mutation subtypes, but also exerts multiplicative synergistic effects with Ivacaftor (HY-13017), and is often used in a triple combination therapy with Tezacaftor (HY-15448). Elexacaftor is widely used in basic and clinical translational research on cystic fibrosis .
    (R)-Elexacaftor
  • HY-157261

    Biochemical Assay Reagents Endocrinology
    UNC2383 is an oligonucleotide enhancer compound. UNC2383 can enhance the efficacy of antisense oligonucleotides (ASOs) and splice-switching oligonucleotides (SSOs). UNC2383 can be used in research of diseases involving impaired oligonucleotide delivery, such as cystic fibrosis .
    UNC2383
  • HY-109187A
    Posenacaftor sodium
    1 Publications Verification

    PTI-801 sodium

    CFTR Inflammation/Immunology
    Posenacaftor (PTI-801) sodium is a cystic fibrosis transmembrane regulator (CFTR) protein modulator that corrects the folding and trafficking of CFTR protein. Posenacaftor sodium is used for the research of cystic fibrosis (CF) .
    Posenacaftor sodium
  • HY-111680
    Nesolicaftor
    2 Publications Verification

    PTI-428

    CFTR Autophagy Endocrinology
    Nesolicaftor (PTI-428) is a specific cystic fibrosis transmembrane conductance regulator (CFTR) amplifier .
    Nesolicaftor
  • HY-109027
    Cavosonstat
    1 Publications Verification

    N91115

    GSNOR CFTR Inflammation/Immunology
    Cavosonstat (N91115) is an orally active S-nitrosoglutathione reductase (GSNOR) inhibitor. Cavosonstat is a CFTR stabilizer, and can be used for cystic fibrosis research .
    Cavosonstat
  • HY-109177

    QBW251

    CFTR Inflammation/Immunology
    Icenticaftor (QBW251) is an orally active CFTR channel potentiator, with EC50s of 79 nM and 497 nM for F508del and G551D CFTR, respectively. Icenticaftor can be used for chronic obstructive pulmonary disease (COPD) and cystic fibrosis research .
    Icenticaftor
  • HY-108464A

    Sodium Channel TRP Channel Metabolic Disease Inflammation/Immunology
    Phenamil methanesulfonate, an analog of Amiloride (HY-B0285), is a more potent and less reversible epithelial sodium channel (ENaC) blocker with an IC50 of 400 nM . Phenamil methanesulfonate is also a competive inhibitor of TRPP3 and inhibits TRPP3-mediated Ca 2+ transport with an IC50 of 140 nM in a Ca 2+ uptake assay . Phenamil methanesulfonate is an intriguing small molecule to promote bone repair by strongly activating BMP signaling pathway . Phenamil methanesulfonate is used for the research of cystic fibrosis lung disease .
    Phenamil methanesulfonate
  • HY-15448A

    (Rac)-VX-661

    CFTR Inflammation/Immunology
    (Rac)-Tezacaftor ((Rac)-VX-661) is a racemate of Tezacaftor (HY-15448). Tezacaftor is a F508del CFTR corrector. (Rac)-Tezacaftor can be used for the research of cystic fibrosis .
    (Rac)-Tezacaftor
  • HY-135304

    Protein Arginine Deiminase Inflammation/Immunology Cancer
    PAD-IN-2 is a potent pad4 inhibitor (IC50: <1 μM). PAD-IN-2 can be used in the research of auto-immune diseases and cancers, such as rheumatoid arthritis, vasculitis, systemic lupus erythematosis, cutaneous lupus erythematosis, ulcerative colitis, cystic fibrosis, asthma, multiple sclerosis and psoriasis .
    PAD-IN-2
  • HY-109152
    Navocaftor
    1 Publications Verification

    GLPG 3067; ABBV-3067

    CFTR Others
    Navocaftor (GLPG 3067), as a cystic fibrosis transmembrane regulator (CFTR), is a protein modulator (US 20200377491 Al, example 1) .
    Navocaftor
  • HY-143344

    PROTACs CFTR Inflammation/Immunology
    NJH-2-056 is a deubiquitinase-targeting chimera (DUBTAC) linking the OTUB1 recruiter EN523 to the CFTR chaperone lumacaftor. NJH-2-056 can be used for cystic fibrosis research .
    NJH-2-056
  • HY-P11085

    Bacterial Infection
    WLBU2 is a engineered cationic antimicrobial peptide (eCAP) that overcomes the environmental sensitivity of natural antimicrobial peptides (AMPs). WLBU2 exhibits rapid bactericidal effect, with the MIC values of ≤ 10 μM against both Gram-negative and Gram-positive bacteria including MRSA, vancomycin-resistant enterococci, K. pneumoniae, E.aerogenes, E. cloacae, Escherichia coli, et, al. WLBU2 prevents P. aeruginosa biofilm growth and retains its activity in an environment rich in mucus, low pH and high salt concentrations without negative effects on human airway epithelial cells. WLBU2 can be used for the studies of cystic fibrosis (CF) and Pseudomonas aeruginosa infections .
    WLBU2
  • HY-119229

    CFTR Inflammation/Immunology
    VRT-325 is a CFTR modulator. VRT-325 inhibits disulfide cross-linking between cysteines in transmembrane segments 6 and 7 of CFTR and P-gp. VRT-325 promotes maturation of CFTR and P-gp processing mutants, rescues ΔF508-CFTR folding at the endoplasmic reticulum. VRT-325 binds ΔF508-CFTR nucleotide-binding domain 1, and increases mature ΔF508-CFTR cell surface expression and chloride conductance. VRT-325 can be used for the research of cystic fibrosis [1] [3].
    VRT-325
  • HY-19970
    KM11060
    2 Publications Verification

    CFTR Autophagy Endocrinology
    KM11060 is a corrector of the F508 deletion (F508del)-cystic fibrosis transmembrane conductance regulator (CFTR) trafficking defect. KM11060 can be used for the research of F508del-CFTR processing defect and development of cystic fibrosis research .
    KM11060
  • HY-112267

    VX-440

    CFTR Autophagy Inflammation/Immunology
    Olacaftor (VX-440) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator extracted from patent US9782408.
    Olacaftor
  • HY-158991

    CFTR Inflammation/Immunology
    I1421 is an activator of the cystic fibrosis transmembrane conductance regulator (CFTR) with an EC50 of 64 nM for WT CFTR currents. I1421 also allosterically activates multiple mutants causing cystic fibrosis (CF) with good in vivo potency, with an oral bioavailability of 60% in mice corresponding to a half-life of 75 min. I1421 synergizes with Elexacaftor (HY-111772) to enhance CFTR currents .
    I1421
  • HY-115878

    CFTR Others
    NJH-2-057 is an EN523 OTUB1 recruiter linked to lumacaftor, a agent used to treat cystic fibrosis that binds ΔF508-CFTR.
    NJH-2-057
  • HY-119936

    CFTR Autophagy Inflammation/Immunology
    GLPG2451 is a cystic fibrosis transmembrane conductance regulator (CFTR) potentiator, which effectively potentiates low temperature rescued F508del CFTR with an EC50 of 11.1 nM .
    GLPG2451
  • HY-P3293

    POL6014

    Elastase Inflammation/Immunology
    Lonodelestat (POL6014) is a potent, orally active and selective peptide inhibitor of human neutrophil elastase (hNE). Lonodelestat (POL6014) has the potential for the research of cystic fibrosis (CF) .
    Lonodelestat
  • HY-135279

    CFTR Inflammation/Immunology
    CFTR corrector 4 (Compound 13), an active (R,R)-form enantiomer, is a highly potent and orally active cystic fibrosis transmembrane conductance regulator (CFTR) corrector. CFTR corrector 4 can increase CFTR levels at the cell surface and have the potential for treatment of cystic fibrosis .
    CFTR corrector 4
  • HY-147249

    CFTR Inflammation/Immunology
    CFTR corrector 8 is a potent CFTR modulator. CFTR can be used in the research of cystic fibrosis .
    CFTR corrector 8
  • HY-Y1422B
    Lipase (MS grade)
    1 Publications Verification

    Endogenous Metabolite Metabolic Disease
    Lipase (MS grade) catalyzes the hydrolysis of triacylglycerols to release long-chain fatty acids in a site-specific manner. Lipase (MS grade) is involved in a variety of biological processes, from fat metabolism to cell signaling and inflammation, and can be used to study diseases such as pancreatic insufficiency, celiac disease and cystic fibrosis .
    Lipase (MS grade)
  • HY-P2835

    Bacterial Infection
    Alginate lyase is a polysaccharide lyase that catalyzes the degradation of alginate. Alginate lyase can be used for the research of cystic fibrosis by degrading the polysaccharide biofilm of Pseudomonas aeruginosa .
    Alginate lyase
  • HY-W984122

    CFTR Inflammation/Immunology
    CFTR corrector 17 is an isoxazole compound and CFTR modulator. CFTR corrector 17 can be used for the research of cystic fibrosis .
    CFTR corrector 17
  • HY-117187

    CFTR Others
    Corr4A is a chemical corrector, which can be used for cystic fibrosis. Corr4A interacts directly with the cystic fibrosis transmembrane conductance regulator (CFTR) or affects indirectly its folding process. Corr4A increases the expression of CFTR ΔF508 on the cell surface, thereby improving its transport to the plasma membrane and increasing the stability of the rescued mutant protein .
    Corr4A
  • HY-169342

    Sodium Channel Inflammation/Immunology
    ETD001 is a long-acting ENaC inhibitor, with an IC50 of 57.5 nM in cultured HBE cells. ETD001 can be used in the study for cystic fibrosis .
    ETD001
  • HY-158144

    CFTR Others
    GLPG2737 is a potent CFTR type 2 corrector, and GLPG2737 can be used in combination with a type 1 co-corrector in the study of cystic fibrosis .
    GLPG2737
  • HY-136939

    CFTR Metabolic Disease Inflammation/Immunology
    CFTR corrector 6 is a potent potentiator of Cystic Fibrosis Transmembrane conductance Regulator (CFTR). CFTR corrector 6 has the potential for cystic fibrosis (CF) and other CFTR associated disorders research .
    CFTR corrector 6
  • HY-120039A

    Elastase Inflammation/Immunology
    (S)-MDL-101146 is the S-isomer of MDL-101146. MDL-101146 is an orally active, competitive and reversible inhibitor against human neutrophil elastase (HNE) with a Ki value of 25 nM. MDL-101146 inhibits HNE-induced hemorrhage in hamsters. MDL-101146 is promising for research of emphysema, rheumatoid arthritis, chronic bronchitis, cystic fibrosis, adult respiratory distress syndrome and glomerulonephritis .
    (S)-MDL-101146
  • HY-145603S

    VX-121-d4

    Isotope-Labeled Compounds CFTR Chloride Channel Neurological Disease
    Vanzacaftor-d4 (VX-121-d4) is the deuterium labeled Vanzacaftor (HY-145603). Vanzacaftor is an orally active noval corrector of Cystic fibrosis transmembrane conductance regulator (CFTR). Vanzacaftor improves processing and trafficking of CFTR protein as well as increases chloride transport in triple combined with Tezacaftor (HY-15448) and Deutivacaftor. Vanzacaftor-Tezacaftor-Deutivacaftor is safe and well tolerated, improving lung function, respiratory symptoms, and CFTR function with cystic fibrosis, which is promising for research in the field of cystic fibrosis diseases .
    Vanzacaftor-d4
  • HY-125381
    CFTR corrector 2
    1 Publications Verification

    CFTR Autophagy Inflammation/Immunology Cancer
    CFTR corrector 2 is a cystic fibrosis transmembrane conductance corrector (CFTR), extracted from patent US20140274933 .
    CFTR corrector 2
  • HY-P3293A

    POL6014 TFA

    Elastase Inflammation/Immunology
    Lonodelestat TFA (POL6014 TFA) is a potent, orally active and selective peptide inhibitor of human neutrophil elastase (hNE). Lonodelestat TFA has the potential for the research of cystic fibrosis (CF) .
    Lonodelestat TFA
  • HY-112267A

    (R)-VX-440

    CFTR Others
    (R)-Olacaftor ((R)-VX-440) is a Cystic fibrosis transmembrane conductance regulator (CFTR) modulator. (R)-Olacaftor has good potential for the study of cystic fibrosis (CF) .
    (R)-Olacaftor
  • HY-114725

    CFpot-532

    CFTR Inflammation/Immunology
    VRT-532 (CFpot-532) is a potent is a potent CFTR modulator. VRT-532 enhances channel activity in G551D-CFTR and intrinsic ATPase activity of G551D-CFTR. VRT-532 has the potential for the research of cystic fibrosis .
    VRT-532
  • HY-164000

    CFTR Others
    CFTR corrector 15 (Compound 4172) is a corrector for cystic fibrosis transmembrane conductance regulator (CFTR), that repairs the F508del-CFTR folding defect when used with VX-809 (HY-13262). CFTR corrector 15 can be used in research of cystic fibrosis disease .
    CFTR corrector 15

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