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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kCAL01
0 ImagesCat. No.: HY-P10806CAS No.: 1364717-03-2kCAL01 is a CAL inhibitor with a Ki value of 2.3 μM. kCAL01 is promising for research of cystic fibrosis (CF). -
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ATX-012
0 ImagesCat. No.: HY-183001CAS No.: 1777792-44-5ATX-012 is an ionizable cationic lipid for the assembly of mRNA-delivering lipid nanoparticles (LNPs). ATX-012 blends with helper lipids, cholesterol and PEG-lipid conjugates to form mRNA-containing nanocarriers, which support efficient cellular internalization and the generation of functional proteins including CFTR. ATX-012 constitutes the vital building block of LNPs applied in cystic fibrosis-related biological studies. -
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Glibenclamide potassium
0 ImagesCat. No.: HY-15206ACAS No.: 52169-36-5Synonyms: Glyburide potassiumGlibenclamide (Glyburide) potassium is a potassium salt of Glibenclamide (HY-15206). Glibenclamid potassium exists in anhydrous and hydrate forms, with higher solubility compared to pure Glibenclamide. -
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Bamocaftor potassium
0 ImagesCat. No.: HY-126394BCAS No.: 2204245-47-4Synonyms: VX-659 potassiumBamocaftor potassium is a cystic fibrosis transmembrane conductance regulator (CFTR) corrector designed to restore F508del-CFTR protein function. Bamocaftor potassium can be used combine with Tezacaftor (HY-15448) and Ivacaftor (HY-13017) in cystic fibrosis research. -
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CP-628006
0 ImagesCat. No.: HY-145126CAS No.: 305822-08-6CP-628006, a small molecule CFTR potentiator, restores ATP-dependent channel gating to the cystic fibrosis mutant G551D-CFTR. -
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Cavosonstat (Standard)
0 ImagesCat. No.: HY-109027RCAS No.: 1371587-51-7Synonyms: N91115 (Standard)Cavosonstat (Standard) is the analytical standard of Cavosonstat (HY-109027). This product is intended for research and analytical applications. Cavosonstat (N91115) is an orally active S-nitrosoglutathione reductase (GSNOR) inhibitor. Cavosonstat is a CFTR stabilizer, and can be used for cystic fibrosis research. -
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Elexacaftor (Standard)
0 ImagesCat. No.: HY-111772RCAS No.: 2216712-66-0Synonyms: VX-445 (Standard)Elexacaftor (Standard) is the analytical standard of Elexacaftor. This product is intended for research and analytical applications. 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. -
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Crinecerfont (Standard)
0 ImagesSynonyms: SSR-125543 (Standard)Crinecerfont (Standard) is the analytical standard of Crinecerfont (HY-106203). This product is intended for research and analytical applications. Crinecerfont (SSR-125543) is an orally effective corticotropin-releasing factor receptor type-1 (CRF1 receptor) antagonist. Crinecerfont blocks CRF1 receptor signaling to reduce adrenocorticotropic hormone secretion. Crinecerfont improves hypothalamic-pituitary-adrenal axis negative feedback sensitivity in chronically stressed mice. Crinecerfont can be used for the research of chronic stress conditions and classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Crinecerfont is a click chemistry reagent, it contains an Alkyne group and can undergo copper-catalyzed azide-alkyne cycloaddition (CuAAc) with molecules containing Azide groups. -
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CFTR(inh)-172 (Standard)
0 ImagesCat. No.: HY-16671RCAS No.: 307510-92-5CFTR(inh)-172 (Standard) is the analytical standard of CFTR(inh)-172 (HY-16671). This product is intended for research and analytical applications. CFTR(inh)-172 is a potent and selective blocker of the CFTR chloride channel; reversibly inhibits CFTR short-circuit current in less than 2 minutes with a Ki of 300 nM. -
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Glibenclamide-13C6
0 ImagesCat. No.: HY-15206S2Synonyms: Glyburide-13C6Glibenclamide-13C6 (Glyburide-13C6) is 13C labeled Glibenclamide. 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. -
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Lumacaftor (Standard)
0 ImagesSynonyms: VX-809 (Standard); VRT 826809 (Standard)Lumacaftor (Standard) is the analytical standard of Lumacaftor. This product is intended for research and analytical applications. Lumacaftor (VX-809; VRT 826809) is a CFTR modulator that corrects the folding and trafficking of CFTR protein. -
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Lubiprostone (Standard)
0 ImagesCat. No.: HY-B0679RCAS No.: 136790-76-6Synonyms: RU-0211 (Standard); SPI-0211 (Standard)Lubiprostone (Standard) is the analytical standard of Lubiprostone. This product is intended for research and analytical applications. Lubiprostone (SPI-0211) increases intestinal fluid secretion through generation of CIC-2/CFTR and activation of cAMP signaling pathway. Lubiprostone inhibits myeloperoxidase (MPO) activity, downregulates Indomethacin (HY-14397)-induced iNOS and TNFα expression. Lubiprostone can be used for chronic constipation research. -
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CFTR activator 2
0 ImagesSynonyms: WAY-326769CFTR activator 2 (WAY-326769) is an activator of mutant cystic fibrosis transmembrane conductance regulator (mutant-CFTR). -
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Steviol (Standard)
0 ImagesSteviol (Standard) is the analytical standard of Steviol (HY-N2057). This product is intended for research and analytical applications. Steviol is the main metabolite of steviol glycosides and an inhibitor of AQP2/CFTR. Steviol slows down the growth of renal cysts by inhibiting the activity of CFTR, reducing the expression of AQP2, and promoting the degradation of AQP2 and CFTR. Steviol can be used in the research of polycystic kidney disease. -
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6-PhEt-dATP
0 ImagesCat. No.: HY-134385CAS No.: 1239610-11-76-PhEt-dATP is an ATP analog that specifically enhances the function of certain CFTR mutants and is useful for studying cystic fibrosis. -
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Ivacaftor-13C6
0 ImagesCat. No.: HY-13017S4Synonyms: VX-770-13C6Ivacaftor-13C6 (VX-770-13C6) is 13C labeled Ivacaftor. Ivacaftor (VX-770) is a potent and orally bioavailable CFTR potentiator, targeting G551D-CFTR and F508del-CFTR with EC50s of 100 nM and 25 nM, respectively. -
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(R)-Posenacaftor sodium (Standard)
0 ImagesCat. No.: HY-109187BRCAS No.: 2095064-09-6Synonyms: (R)-PTI-801 sodium (Standard)(R)-Posenacaftor sodium (Standard) is the analytical standard of (R)-Posenacaftor (sodium) (HY-109187B). This product is intended for research and analytical applications. (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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Ataluren-13C6
0 ImagesCat. No.: HY-14832S1Synonyms: PTC124-13C6Ataluren-13C6 (PTC124-13C6) is 13C labeled Ataluren. Ataluren (PTC124) is an orally available CFTR-G542X nonsense allele inhibitor. -
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PGD97
0 ImagesCat. No.: HY-P10226CAS No.: 2717490-36-1PGD97 is a selective cyclic peptide inhibitor against CAL/CFTR interactions, with a KD value of 6 nM towards the CAL PDZ domain for its desulfide cyclized form. PGD97 (desulfide cyclized form) has selectivity ≥ 130-fold compared to NHERF1/2 PDZ domains. PGD97 is capable of stabilizing F508del-CFTR at the cell membrane and improving CFTR function required for proper fluid homeostasis in tne lung. PGD97 can be used for the research of cystic fibrosis. -
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Galicaftor (Standard)
0 ImagesCat. No.: HY-111111RCAS No.: 1918143-53-9Synonyms: ABBV-2222 (Standard); GLPG-2222 (Standard)Galicaftor (Standard) is the analytical standard of Galicaftor (HY-111111). This product is intended for research and analytical applications. 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. -
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