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| 货号 | 产品名 | 中文名称 | 产品描述 |
|---|---|---|---|
| SJ-MX0768 | Ataluren (PTC124) | 阿塔鲁伦 |
Ataluren (PTC124) 是可口服的 CFTR-G542X 无义等位基因抑制剂。它选择性诱导核糖体转录通读，但不影响正常的终止密码子，在HEK293细胞中EC50为0.1 μM，可以治疗nonsense mutations (如CFTR无义突变引起的CF)造成的家族遗传性疾病，比如囊性纤维化。 |
| SJ-MX0349 | VX-809 (Lumacaftor) | 鲁玛卡托 |
VX-809 (Lumacafto，VRT 826809) 是是一种有效的选择性CFTR调节剂，通过促进突变型CFTR(F508del-CFTR)的成熟，从而纠正囊性纤维症中常见的CFTR突变，在fisher大鼠甲状腺细胞中EC50为0.1 μM。 |
| SJ-MX3391 | PPQ-102 | PPQ -102 is a potent, voltage-independent CFTR chloride channel inhibitor (IC50 = 90 nM). PPQ-102 displays nephroprotective and anti-fibrotic activities, decreasing the size of pre-formed kidney cysts in animal models of polycystic kidney disease. | |
| SJ-MX3582 | GlyH-101 | CFTR Inhibitor II, or GlyH-101, is a glycine hydrazide that selectively and reversibly blocks the CFTR channel (Ki = 4.3 µM). GlyH-101 binds to a site at the external pore of CFTR, occluding the pore and rapidly preventing chloride transport. | |
| SJ-MX1956 | Nesolicaftor (PTI-428 ) | Nesolicaftor (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。 | |
| SJ-MX0099 | Elexacaftor(VX-445) | Elexacaftor (VX-445， Compound 1) 是一种囊性纤维化跨膜电导调节因子 (CFTR) 校正剂。Elexacaftor (VX-445， Compound 1) 促进CFTR的加工和转运，增加细胞表面CFTR的数量。 | |
| SJ-MX1953 | GLPG1837 | 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。 | |
| SJ-MX0198 | Ivacaftor (VX-770) | 依伐卡托 |
Ivacaftor (VX-770)是口服生物可利用的 CFTR 增强剂；靶向作用于G551D-CFTR和F508del-CFTR，在fisher大鼠甲状腺细胞中EC50分别为100 nM和25 nM。Ivacaftor被FDA 批准的用于临床治疗囊性纤维化 (CF) 患者的药物。 |
| SJ-MX2899 | IOWH-032 | IOWH-032 (IOWH032; IOWH 032) is a potent and synthetic CFTR (Cystic fibrosis transmembrane conductance regulator) inhibitor with antifibrotic activity. It inhibits CFTR with IC50s of 1.01 μM and in CHO-CFTR and T84-CFTR cell based assays, respectively. IOWH-032 rapidly blocked and potentiated hCFTR activity in a concentration-dependent manner with apparent Kd value of 6.1 nM and 0.64 nM, respectively. However, IOWH-032 did not potentiate and only blocked mCFTR with an apparent Kd value of 42.9 μM. | |
| SJ-MX3618 | KM11060 | KM11060 is a novel mutated corrector of the F508del-CFTR (cystic fibrosis transmembrane conductance regulator) trafficking defect. It corrects F508del-CFTR trafficking, and increases the amount of functional CFTR at the plasma membrane (~75%) and inhibits PDE5 activity. Small-molecule correctors such as KM11060 may serve as useful pharmacological tools in studies of the F508del-CFTR processing defect and in the development of cystic fibrosis therapeutics. KM11060 partially corrects F508del-CFTR processing and increases surface expression to 75% of that observed in cells incubated at low temperature. Up to 50% of the F508del-CFTR in cells treated with KM11060 was complex-glycosylated, indicating passage through the Golgi. KM11060 as a promising compound for further development of CF therapeutics. | |
| SJ-MX0695 | CFTRinh-172 |
CFTRinh-172 (CFTR inhibitor 172) 是高效选择性的、电势差不依赖性的选择性CFTR抑制剂，在2分钟内可逆地抑制CFTR短路电流的 Ki 值为300 nM。CFTRinh-172对于MDR1，ATP敏感性钾离子通道或者一系列的转运蛋白没有效果。 |
|
| SJ-MN0514 | Glyburide (Glibenclamide) | 格列本脲 |
Glyburide (Glibenclamide)是一种具有口服活性的 ATP 敏感的 K+ 通道 (KATP) 抑制剂，可用于糖尿病和肥胖的研究。Glyburide抑制 P-glycoprotein。Glyburide直接结合并阻断KATP 的 SUR1 亚基并抑制囊性纤维化跨膜传导调节蛋白 (CFTR)。Glibenclamid 可诱导自噬（autophagy）。 |
| SJ-MX4168 | CFTR corrector 2 | CFTR corrector 2 (FDL-169) is a novel and potent CFTR (Cystic fibrosis transmembrane conductance regulator) corrector being developed by Flatley Discovery Lab for treating cystic fibrosis (CF) patients who carry the F508del mutation, the most common mutation in this disease. | |
| SJ-MX4976 | Icenticaftor | ||
| SJ-MX0156 | Tezacaftor (VX-661) | 替扎卡托 | Tezacaftor (VX-661) 是 F508del CFTR 的矫正器，能够帮助 CFTR 蛋白与细胞表面结合。 |
| SJ-MX5669 | (R)-BPO-27 |
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