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  1. Membrane Transporter/Ion Channel
  2. CFTR

CFTR

CFTR (Cystic fibrosis transmembrane conductance regulator) is a membrane protein in vertebrates that is encoded by the CFTR gene. CFTR is an ABC transporter-class ion channel that conducts chloride and thiocyanate ions across epithelial cell membranes. Mutations of the CFTR gene affecting chloride ion channel function lead to dysregulation of epithelial fluid transport in the lung, pancreas and other organs, resulting in cystic fibrosis. Complications include thickened mucus in the lungs with frequent respiratory infections, and pancreatic insufficiency giving rise to malnutrition and diabetes. These conditions lead to chronic disability and reduced life expectancy. CFTR functions as a cAMP-activated ATP-gated anion channel, increasing the conductance for certain anions to flow down their electrochemical gradient. ATP-driven conformational changes in CFTR open and close a gate to allow transmembrane flow of anions down their electrochemical gradient. CFTR is an ion channel that evolved as a 'broken' ABC transporter that leaks when in open conformation.

CFTR 相关产品 (15):

Cat. No.Product NameEffectPurity
  • HY-13017
    IvacaftorAgonist 99.75%
    Ivacaftor是一种有效可口服的 CFTR增效剂,靶向G551D-CFTR和F508del-CFTR的EC50分别为100 nM 和 25 nM。
  • HY-13262
    LumacaftorModulator 99.12%
    Lumacaftor (VX-809) 是 CFTR 调节剂,可纠正 CFTR 蛋白的折叠和运输。
  • HY-14832
    AtalurenInhibitor 99.88%
    Ataluren (PTC124) 是可口服的 CFTR-G542X 无义等位基因抑制剂。
  • HY-16671
    CFTR(inh)-172Inhibitor 98.82%
    CFTR(inh)-172是高效,选择性的 CFTR 通道阻断剂; 在2分钟内可逆地抑制CFTR短路电流的 Ki 值为300 nM。
  • HY-15448
    TezacaftorModulator 99.70%
    Tezacaftor (VX-661) 是 F508del CFTR 的矫正器,能够帮助 CFTR 蛋白与细胞表面结合。
  • HY-111680
    PTI-428Modulator 99.77%
    PTI-428 是一种特异性囊性纤维化跨膜传导调节因子 (CFTR) 调节剂。
  • HY-18336
    GlyH-101Inhibitor 99.35%
    GlyH-101是可渗透入细胞的CFTR阻断剂,Ki为1.4 uM。
  • HY-19778
    (R)-BPO-27Inhibitor 99.56%
    (R)-BPO-27是有效的CFTR抑制剂,IC50值为4 nM。
  • HY-111099
    GLPG1837Agonist 99.15%
    GLPG1837 是一种有效的,可逆的 CFTR 增强剂,对 CFTR 的 突变体 F508del 和 G551D 的 EC50 值分别为 3 nM 和 339 nM。
  • HY-14179
    PPQ-102Inhibitor 98.19%
    PPQ-102是CFTR抑制剂,能抑制CFTR氯流,IC50为90 nM。
  • HY-19778A
    BPO-27 racemateInhibitor 98.67%
    BPO-27 racemate是有效的CFTR抑制剂,IC50值为8 nM。
  • HY-13017A
    Ivacaftor benzenesulfonateAgonist
    Ivacaftor benzenesulfonate 是一种可口服的 CFTR 增强剂,常用于囊性纤维化的治疗。
  • HY-13017B
    Ivacaftor hydrateAgonist
    Ivacaftor hydrate 是一种可口服的 CFTR 增强剂,常用于囊性纤维化的治疗。
  • HY-18337
    IOWH-032Inhibitor 99.66%
    IOWH-032是CFTR抑制剂,在T84和CHO-CFTR细胞实验中IC50为1.01 uM。
  • HY-19970
    KM11060Activator 99.80%
    KM11060是F508del-CFTR校正剂。
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