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Cystic fibrosis diseases

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34

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2

Biochemical Assay Reagents

2

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2

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2

Isotope-Labeled Compounds

Targets Recommended:
Cat. No. Product Name Target Research Areas Chemical Structure
  • 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-B1153
    Glafenine
    4 Publications Verification

    Glafenin

    		 COX CFTR Apoptosis Endoplasmic Reticulum Oxidoreductase 1 (ERO1) Metabolic Disease Inflammation/Immunology
    Glafenine (Glafenin) is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF) .
    Glafenine
  • HY-N2388
    Auraptene

    		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-112624J
    Dextran T4 (MW 4,000)

    Dextran 4; Dextran D4; Dextran T4(MW 3200-4800)

    		 Bacterial Others
    Dextran 4,000 is a mucus rheology modifier. The dextran molecules in Dextran 4,000 can reduce the cross-link density of mucus through osmotic effects and hydrogen bond substitution, and reduce viscoelasticity and improve the mucociliary/cough clearance index by destroying the DNA-mucin network structure in mucus. Dextran 4,000 has the ability to improve the rheological properties and clearance ability of cystic fibrosis (CF) sputum, and can be used in the study of inhalation therapy or aerosol delivery of mucostatic respiratory diseases. The Dextran series of compounds are also natural polysaccharide drug carriers that can be connected to drugs through covalent bonding methods such as ester bonds, amide bonds or click chemistry, or self-assembled to form carriers such as nanoparticles and hydrogels. Dextran is biodegradable and biocompatible, and can achieve targeted delivery and controlled release of drugs. Dextran derivatives can prolong the half-life of drugs, increase local concentrations, and reduce the activity of immune clearance .
    Dextran T4 (MW 4,000)
  • HY-108882B
    DNase I RNase & Protease free
    Maximum Cited Publications
    29 Publications Verification

    		 DNA/RNA Synthesis Inflammation/Immunology
    DNase I is an enzyme that degrades DNA. DNase I is mainly produced by digestive system organs, such as the pancreas and parotid gland. Three types of DNase I are known in mammals: pancreatic type, parotid type, and pancreatico-parotid type. DNase I plays a key role in the cleavage of extracellular DNA, and is critical for limiting inflammatory responses and maintaining homeostasis. DNase I is responsible for digesting extracellular nucleoproteins, which may be essential for preventing autoimmune responses. Decreased DNase I activity may be associated with the onset and progression of systemic lupus erythematosus (SLE). DNase I (RNase & Protease free) is a molecular biology-grade DNase I purified by chromatography to remove RNase and protease .
    DNase I RNase & Protease free
  • HY-157261
    UNC2383

    		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-103370
    Talniflumate

    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-108464A
    Phenamil methanesulfonate

    		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-109177
    Icenticaftor

    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-135304
    PAD-IN-2

    		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-B1153A
    Glafenine hydrochloride
    4 Publications Verification

    Glafenin hydrochloride

    		 COX CFTR Apoptosis Endoplasmic Reticulum Oxidoreductase 1 (ERO1) Metabolic Disease Inflammation/Immunology
    Glafenine (Glafenin) hydrochloride is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine hydrochloride exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine hydrochloride can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine hydrochloride can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF) .
    Glafenine hydrochloride
  • HY-P4846
    Ac-Pro-Gly-Pro-OH
    2 Publications Verification

    		 CXCR Apoptosis IFNAR TNF Receptor Interleukin Related Infection Inflammation/Immunology
    Ac-Pro-Gly-Pro-OH is an endogenous degradation product of extracellular collagen and acts as a CXCR2 agonist . Ac-Pro-Gly-Pro-OH exerts bactericidal activity by generating hydrogen peroxide, inhibits pulmonary inflammation, and reduces immune cell apoptosis (apoptosis). Ac-Pro-Gly-Pro-OH promotes the production of IFN-γ and inhibits the production of TNF-α and IL-6 in leukocytes. Ac-Pro-Gly-Pro-OH increases the survival rate of mice in sepsis models, enhances the bactericidal activity of neutrophils, acts as a neutrophil chemoattractant, induces neutrophil polarization, and regulates inflammatory and repair processes. Ac-Pro-Gly-Pro-OH induces chronic inflammation and tissue remodeling through sustained action. Ac-Pro-Gly-Pro-OH is released via alkaline hydrolysis of corneal proteins in alkali-injured eyes, thereby driving the early infiltration of neutrophils into the cornea. Ac-Pro-Gly-Pro-OH is applicable to research related to sepsis, chronic obstructive pulmonary disease, cystic fibrosis, bronchiolitis obliterans syndrome, severe asthma, idiopathic pulmonary fibrosis, and corneal ulcer .
    Ac-Pro-Gly-Pro-OH
  • 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-B1153R
    Glafenine (Standard)

    Glafenin (Standard)

    		 COX CFTR Apoptosis Endoplasmic Reticulum Oxidoreductase 1 (ERO1) Reference Standards Metabolic Disease Inflammation/Immunology
    Glafenine (Standard) is the analytical standard of Glafenine. This product is intended for research and analytical applications. Glafenine (Glafenin) is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF) .
    Glafenine (Standard)
  • HY-145603S
    Vanzacaftor-d4

    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-179041
    SZ0232

    		PGE synthase Prostaglandin Receptor MDM-2/p53 Amino acid Transporter Glutathione Peroxidase Ferroptosis Metabolic Disease Endocrinology
    SZ0232 is a selective mPGES-2 inhibitor. SZ0232 binds to the active site of mPGES-2 via hydrogen bonds and π-π stacking, reduces the production of prostaglandin E2 (PGE2) and blocks the PGE2-EP3 pathway. SZ0232 regulates Ferroptosis by activating the heme-dependent p53/SLC7A11/GPX4 axis, inhibits lipid peroxidation, and protects renal tubules. SZ0232 enhances glucose-stimulated insulin secretion, inhibits β-cell senescence, and improves glucose homeostasis. SZ0232 reduces renal lipid accumulation, alleviates fibrosis, and ameliorates renal dysfunction in diabetic mice. SZ0232 inhibits renal cyst growth in polycystic kidney disease models. SZ0232 exhibits an insulinotropic effect that strengthens with the increase of animal age. SZ0232 can be used in studies related to type 2 diabetes, acute kidney injury, diabetic kidney disease, and autosomal dominant polycystic kidney disease .
    SZ0232
  • HY-164000
    CFTR corrector 15

    		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
  • HY-106293
    ICI-200880

    		Elastase Inflammation/Immunology
    ICI-200880 is a potent, selective and reversible human neutrophil elastase (HNE) inhibitor. ICI-200880 is promising for research of inflammatory lung diseases related to neutrophil elastase, such as chronic obstructive pulmonary disease (COPD) and cystic fibrosis (CF) .
    ICI-200880
  • HY-112363
    Aloisine A

    RP107

    		 CDK GSK-3 ERK JNK CFTR Inflammation/Immunology Cancer
    Aloisine A (RP107) is a a potent cyclin-dependent kinase (CDK) inhibitor with IC50s of 0.15 μM, 0.12 μM, 0.4 μM, 0.16 μM for CDK1/cyclin B, CDK2/cyclin A, CDK2/cyclin E, CDK5/p35, respectively. Aloisine A ininhibits GSK-3α (IC50=0.5 μM) and GSK-3β (IC50=1.5 μM). Aloisine A stimulates wild-type CFTR and mutated CFTR, with submicromolar affinity by a cAMP-independent mechanism. Aloisine A has the potential for CFTR-related diseases, including cystic fibrosis research .
    Aloisine A
  • HY-169000
    CFTR corrector 16

    		CFTR Others
    CFTR corrector 16 (Compound 39) is a corrector for cystic fibrosis transmembrane conductance regulator (CFTR), that can be used in research of cystic fibrosis disease .
    CFTR corrector 16
  • HY-137607A
    UMP-CP trisodium

    		P2Y Receptor Inflammation/Immunology
    UMP-CP trisodium (Compound 3), a nucleotide, is a P2Y14 receptor agonist with an EC50 of 160  nM for P2Y14 receptor over the P2Y6 receptor. UDP trisodium can be used for pain, diabetes, cystic fibrosis and other pulmonary diseases research .
    UMP-CP trisodium
  • HY-161894
    CFTR corrector 13

    		CFTR Inflammation/Immunology
    CFTR corrector 13 (SVQ18) is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector (EC50=3.14 μM). CFTR corrector 13 enhances the function of CFTR channels, especially when used in combination with Lumacaftor (HY-13262), it can produce a dose-dependent increase in CFTR function. CFTR corrector 13 can be used in the study of cystic fibrosis (CF) and other CFTR-related diseases .
    CFTR corrector 13
  • HY-151164
    LasR-IN-2

    		Bacterial Infection
    LasR-IN-2 is a LasR inhibitor that forms H-bonding with TRY-56 residue. LasR-IN-2 can be used in the research of bacterial infection, neutropenia, severe burns and chronic lung disease in cystic fibrosis (CF) .
    LasR-IN-2
  • HY-162496
    L-NBDNJ

    		Bacterial Infection Inflammation/Immunology
    L-NBDNJ, a glycomimetic, is an antivirulence agent. L-NBDNJ interferes with the expression of proteins regulating cytoskeleton assembly and organization of the host cell. L-NBDNJ has anti-inflammatory and anti-infective effects in models of cystic fibrosis (CF) lung disease infection .
    L-NBDNJ
  • HY-115676
    GSK199 analog hydrochloride

    		Protein Arginine Deiminase Inflammation/Immunology
    GSK199 analog hydrochloride belongs to a class of compounds that inhibit PAD4 (guanidinoarginine deiminase 4). GSK199 analog hydrochloride has potential uses in diseases including rheumatoid arthritis, vasculitis, systemic lupus erythematosus, ulcerative colitis, cancer, cystic fibrosis, asthma, cutaneous lupus erythematosus, and psoriasis .
    GSK199 analog hydrochloride
  • HY-N2388R
    Auraptene (Standard)

    		Reference Standards MMP PPAR Bacterial Cardiovascular Disease Neurological Disease Inflammation/Immunology Cancer
    Auraptene (Standard) is the analytical standard of Auraptene. This product is intended for research and analytical applications. 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 (Standard)
  • HY-15448R
    Tezacaftor (Standard)

    VX-661 (Standard)

    		 CFTR Reference Standards Cancer
    Tezacaftor (Standard) is the analytical standard of Tezacaftor. This product is intended for research and analytical applications. 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 (Standard)
  • HY-15448S1
    Tezacaftor-d6

    VX-661-d6

    		 Isotope-Labeled Compounds CFTR Cancer
    Tezacaftor-d6 (VX-661-d6) is deuterium labeled Tezacaftor. 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-d6
  • HY-162033
    PAT1inh-A0030

    		Chloride Channel Others
    PAT1inh-A0030 is a selective PAT1 (SLC26A6) inhibitor (IC50= 1.0 μM). PAT1inh-A0030 inhibits fluid absorption in the ileum of wild-type and cystic fibrosis (CF) mice (CftrdelF508/delF508) in a closed-loop model of intestinal fluid absorption. PAT1inh-A0030 can be used in the study of intestinal diseases related to CF .
    PAT1inh-A0030
  • HY-W127461
    Ganglioside GM2, Asialo

    Gangliotriosylceramide

    		 Biochemical Assay Reagents Others
    Ganglioside GM2 asialo (asialo-GM2) is a glycosphingolipid containing three monosaccharide residues and one fatty acid of variable chain length, but lacks the sialic acid residue present on ganglioside M2. Asialo-GM2 is found at low or undetectable levels in normal human brains, but it accumulates in the brains of patients with Tay-Sachs disease and Sandhoff disease, which are expressed as lysosomal β- A neurodegenerative disorder characterized by hexosaminidase A and B deficiency. It also binds to various bacteria, including Pseudomonas isolated from cystic fibrosis patients. The Asialo-GM2 mixture contains ganglioside GM2 asialo molecular species with fatty acyl chains of variable length.
    Ganglioside GM2, Asialo
  • HY-B1153AR
    Glafenine hydrochloride (Standard)

    Glafenin hydrochloride (Standard)

    		 COX CFTR Apoptosis Endoplasmic Reticulum Oxidoreductase 1 (ERO1) Reference Standards Metabolic Disease Inflammation/Immunology
    Glafenine (hydrochloride) (Standard) is the analytical standard of Glafenine (hydrochloride). This product is intended for research and analytical applications. Glafenine (Glafenin) hydrochloride is a non-selective, non-steroidal anti-inflammatory drug-based COX-1/COX-2 inhibitor. Glafenine hydrochloride exerts anti-inflammatory, anti-proliferative and anti-cell migration effects by inhibiting the arachidonic acid metabolic pathway and reducing prostaglandin synthesis. Glafenine hydrochloride can induce cell cycle arrest in vascular smooth muscle cells and endothelial cells and reduce the synthesis of the extracellular matrix protein Tenascin. Glafenine hydrochloride can be used in the research of inflammatory-related diseases, vascular restenosis and cystic fibrosis (CF) .
    Glafenine hydrochloride (Standard)
  • HY-103370R
    Talniflumate (Standard)

    BA 7602-06 (Standard)

    		 Reference Standards Chloride Channel Inflammation/Immunology
    Talniflumate (Standard) is the analytical standard of Talniflumate. This product is intended for research and analytical applications. 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 Ca2+-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 (Standard)
  • HY-113500A
    Hepoxilin A3

    HXA3

    		 Lipoxygenase Inflammation/Immunology
    Hepoxilin A3 (HXA3) is a neutrophil chemo-attractant, synthesized by activating the PLA2-12-LOX pathway. Hepoxilin A3 can guide neutrophils to cross the epithelial barrier and migrate to the infection site (such as the alveolar cavity). The level of Hepoxilin A3 increases synchronously with neutrophil infiltration in mouse models. Hepoxilin A3 can be used to study inflammatory diseases (such as pneumonia, cystic fibrosis) .
    Hepoxilin A3

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