| 应用 |
Is a CFTR activator |
| 产品介绍 |
Ivacaftor (VX-770)是一种囊性纤维化跨膜传导调节器(CFTR)的增强剂，靶向作用于G551D-CFTR和F508del-CFTR，EC50分别为100 nM和25 nM。 |
| 备注 |
Ivacaftor (VX-770, Kalydeco) is a potentiator of CFTR targeting G551D-CFTR and F508del-CFTR with EC50 of 100 nM and 25 nM, respectively. |
| 生化机理 |
Ivacaftor is a CFTR activator (IC50 of 43 ± 38 nM). In some cells Ivacaftor was shown to increase CFTR channel open probability in the F508del processing and the G551D gating mutations. In cultured human CF bronchial epithelia (HBE) expressing the F551D gating mutation on one allele and F508del processing mutation on the other allele, Ivacaftor was shown to increase Cl- secretion by about 10-fold, to about 50% of that observed in HBE isolated from individuals without CF, in addition, Ivacaftor reduced excessive Na+ and fluid absorption preventing dehydration of the apical surface and increased cilia beating in these epithelial cultures. |
| 别名 |
VX 770; N-(2,4-二叔丁基-5-羟基苯基)-1,4-二氢-4-氧代-3-喹啉甲酰胺;VX770; Ivacaftor; VX 770;Kalydeco;N-[2,4-Bis(tert-butyl)-5-hydroxyphenyl]-1,4-dihydro-4-oxo-3-quinolinecarboxamide |
