CFTR
Cystic fibrosis transmembrane conductance regulator
CFTR (Cystic fibrosis transmembrane conductance regulator), mutations of which cause cystic fibrosis, belongs to the ATP-binding cassette (ABC) transporter family and works as a channel for small anions, such as chloride and bicarbonate. CFTR is composed of two homologous halves, each comprising a transmembrane (TMD) and a nucleotide binding domain (NBD). CFTR activity is regulated by phosphorylation of its cytosolic regulatory (R) domain, and ATP binding and hydrolysis at two NBDs.
CFTR is expressed in many cell types throughout the body, but in the airways it is found mainly in secretory serous cells of the submucosal glands. Transitions between open and closed states of CFTR are regulated by ATP binding and hydrolysis on the cytosolic nucleotide binding domains, which are coupled with the transmembrane (TM) domains forming the pathway for anion permeation. CFTR function is normally tightly controlled as dysregulation can lead to life-threatening diseases such as secretory diarrhoea and cystic fibrosis.
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CFTR Related Products (133)
Related Products (133)
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Antibodies (1)
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Vanzacaftor-d4
0 ImagesCat. No.: HY-145603SSynonyms: VX-121-d4Vanzacaftor-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.
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Ataluren (Standard)
0 ImagesSynonyms: PTC124 (Standard)Ataluren (Standard) is the analytical standard of Ataluren. This product is intended for research and analytical applications. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor.
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Corr4A
0 ImagesCorr4A 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.
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NVS1.1
0 ImagesCat. No.: HY-177569NVS1.1 is an orally active, blood-brain barrier-penetrant eRF1 degrader. NVS1.1 induces ubiquitination of eRF1 at Lys279, mediates proteasomal degradation via the E3 ubiquitin ligases RNF14 and RNF25 as well as the translational stress sensor GCN1, traps eRF1 at the ribosomal A-site, inhibits translation termination and triggers ribosome collision. As a readthrough enhancer, NVS1.1 enables near-cognate tRNA incorporation at premature termination codons by reducing intracellular eRF1 levels. NVS1.1 activates ribosome-associated quality control pathways via ribosome collision, including ubiquitination of small subunit ribosomal proteins. NVS1.1 restores functional full-length CFTR and IDUA proteins and reduces glycosaminoglycan accumulation in relevant models. NVS1.1 can be used in the research of cystic fibrosis and Hurler syndrome (mucopolysaccharidosis type I, MPS I).
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(R)-Posenacaftor sodium
0 ImagesSynonyms: (R)-PTI-801 sodium
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Kobusin
0 ImagesKobusin is a bisepoxylignan isolated from the Pnonobio biondii Pamp. Kobusin is an activator of CFTR and CaCCgie chloride channels and a inhibitor of ANO1/CaCC (calcium-activated chloride channel) channel.
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H2-Gamendazole
0 ImagesH2-Gamendazole is a derivative of Lonidamine that reduces cyst formation in polycystic kidney disease and is used in autosomal dominant polycystic kidney disease research.
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PG01
0 ImagesPG01 is a potent CFTR Cl- channel potentiator. PG01 can correct gating defects of CFTR mutants, is effective on b>E193K, G970R and G551D (CFTR mutants) with Kd values of 0.22 μM, 0.45 μM and 1.94 μM, respectively. PG01 is also effective on ΔF508 (Ka of 0.3 μM). PG01 increases ΔF508-CFTR Cl- current after adding Forskolin.
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Ivacaftor benzenesulfonate
0 ImagesCat. No.: HY-13017ACAS No.: 1134822-09-5Synonyms: VX-770 benzenesulfonateIvacaftor benzenesulfonate is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
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Ivacaftor hydrate
0 ImagesCat. No.: HY-13017BCAS No.: 1134822-07-3Synonyms: VX-770 hydrateIvacaftor hydrate (VX-770 hydrate) is an orally bioavailable CFTR potentiator, used for cystic fibrosis treatment.
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2-Amino-2-deoxy-D-glucopyranose
0 ImagesCat. No.: HY-W615152CAS No.: 90-77-72-Amino-2-deoxy-D-glucopyranose is an isomeric form of Glucosamine (HY-B1125). 2-Amino-2-deoxy-D-glucopyranose is an amino sugar and a prominent precursor in the biochemical synthesis of glycosylated proteins and lipids, is used as a dietary supplement. 2-Amino-2-deoxy-D-glucopyranose also is a natural constituent of glycosaminoglycans in the cartilage matrix and synovial fluid, which when administered exogenously, exerts pharmacological effects on osteoarthritic cartilage and chondrocytes.
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5,7,4'-Trimethoxyflavone (Standard)
0 Images5,7,4'-Trimethoxyflavone (Standard) is the analytical standard of 5,7,4'-Trimethoxyflavone. This product is intended for research and analytical applications. 5,7,4’-Trimethoxyflavone can be isolated from the medicinal plant Kaempferia parviflora (KP). 5,7,4’-Trimethoxyflavone is a CFTR activator and EC50 is 64 μM. 5,7,4’-Trimethoxyflavone induces apoptosis, increases proteolytic activation of caspase-3, and degradation of ADP-ribose polymerase (PARP) protein. 5,7,4’-Trimethoxyflavone has antitumor activity. 5,7,4’-Trimethoxyflavone can be used to prevent skin aging and oxidative stress.
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GLPG-3221
0 ImagesCat. No.: HY-133013CAS No.: 2222264-64-2GLPG-3221 is a potent, orally active corrector of CFTR (cystic fibrosis transmembrane conductance regulator), with an EC50 of 105 nM. GLPG-3221 can be uesd for the treatment of cystic fibrosis.
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Posenacaftor
0 ImagesCat. No.: HY-109187CAS No.: 2095064-05-2Synonyms: PTI-801
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CFTR corrector 16
0 ImagesCat. No.: HY-169000CAS No.: 1210305-23-9
