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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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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CFTR corrector 4
0 ImagesCFTR 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.
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CFTR corrector 2
0 ImagesCFTR corrector 2 is a cystic fibrosis transmembrane conductance corrector (CFTR), extracted from patent US20140274933.
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CFTR corrector 17
0 ImagesCFTR corrector 17 is an isoxazole compound and CFTR modulator. CFTR corrector 17 can be used for the research of cystic fibrosis.
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(R)-Posenacaftor sodium
0 ImagesSynonyms: (R)-PTI-801 sodium(R)-Posenacaftor (R)-PTI-801) sodium is the R enantiomer of Posenacaftor. Posenacaftor is a cystic fibrosis transmembrane regulator (CFTR) protein modulator that corrects the folding and trafficking of CFTR protein. Posenacaftor is used for the research of cystic fibrosis (CF).
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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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CFTR corrector 8
0 ImagesCat. No.: HY-147249CAS No.: 1918142-35-4
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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-801Posenacaftor (PTI-801) is a cystic fibrosis transmembrane regulator (CFTR) protein modulator that corrects the folding and trafficking of CFTR protein. Posenacaftor is used for the research of cystic fibrosis (CF).
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CFTR corrector 16
0 ImagesCat. No.: HY-169000CAS No.: 1210305-23-9CFTR corrector 16 (Compound 39) is a corrector for cystic fibrosis transmembrane conductance regulator (CFTR), that can be used in research of cystic fibrosis disease.
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ARN23765
0 ImagesCat. No.: HY-133569CAS No.: 2244458-40-8ARN23765 is a corrector for F508del-CFTR with an EC50 of 38 pM in human bronchial epithelial cells. ARN23765 improves the maturation and function of F508del-CFTR on the cell membrane, affects the ions transport and secretion, and corrects the pathological mechanism of cystic fibrosis (CF).
