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 (13):

Cat. No. Product Name Effect Purity
  • HY-13262
    VX-809 Modulator 99.12%
    VX-809 是一种 CFTR 校正药物,促进 F508del-CFTR 成熟,EC50 为 0.1±0.1 μM。
  • HY-13017
    Ivacaftor Agonist 98.36%
    Ivacaftor 是一种可口服的 CFTR 的增强剂,常用于囊性纤维化的治疗。
  • HY-14832
    PTC124 Inhibitor 99.88%
    PTC124靶向作用于无义突变,将终止密码子插入到CFTR基因中间。
  • HY-16671
    CFTR(inh)-172 Inhibitor 98.87%
    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
    VX-661 Modulator 99.56%
    VX-661 是 F508del CFTR 的矫正器,能够帮助 CFTR 蛋白与细胞表面结合。
  • HY-18336
    GlyH-101 Inhibitor
    GlyH-101是可渗透入细胞的CFTR阻断剂,Ki为1.4 uM。
  • HY-19778A
    BPO-27 racemate Inhibitor 98.67%
    BPO-27 racemate是有效的CFTR抑制剂,IC50值为8 nM。
  • HY-19778
    (R)-BPO-27 Inhibitor
    (R)-BPO-27是有效的CFTR抑制剂,IC50值为4 nM。
  • HY-18337
    IOWH-032 Inhibitor
    IOWH-032是CFTR抑制剂,在T84和CHO-CFTR细胞实验中IC50为1.01 uM。
  • HY-14179
    PPQ-102 Inhibitor 98.19%
    PPQ-102是CFTR抑制剂,能抑制CFTR氯流,IC50为90 nM。
  • HY-19970
    KM11060 Activator
    KM11060是F508del-CFTR校正剂。
  • HY-13017A
    Ivacaftor benzenesulfonate Agonist
    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 hydrate Agonist
    Ivacaftor hydrate 是一种可口服的 CFTR 增强剂,常用于囊性纤维化的治疗。