Ivacaftor (also known as Kalydeco or VX-770 ) is a drug used for the management of Cystic Fibrosis (CF). It is manufactured and distributed by Vertex Pharmaceuticals. It was approved by the Food and Drug Administration on January 31, 2012, and by Health Canada in late 2012.
447 rows · Ivacaftor (also known as Kalydeco or VX-770 ) is a drug used for the management of.
6/17/2019 · The recent approval of the CFTR potentiator drug ivacaftor ( Vx-770 ) for the treatment of CF patients has marked the advent of causative CF therapy. Currently, thousands of patients are being treated with the drug, and its molecular mechanism of action .
3/1/2013 · Ivacaftor ( VX-770 Vertex Pharmaceuticals, Cambridge, … as a proof-of-concept of the drug mechanism of action . There were no important safety concerns for >48 weeks administration. Initial analysis of an ongoing longer term open-label study suggests that the efficacy of ivacaftor will be maintained in the long-term.
Our current understanding for the action of VX-770 can be summarized in three major features: first, VX-770 increases both ATP-dependent and ATP-independent gating of CFTR (Eckford et al.
2012 Jih and Hwang, 2013) second, VX-770 slows down nonhydrolytic closing (Jih and Hwang, 2013 Kopeikin et al.
2014) third, VX -770 potentiates mutants with defective NBD dimerization .
11/3/2009 · The direct action of VX-770 on CFTR is supported by studies indicating that VX-770 did not modulate cAMP/PKA signaling (Fig. 1F). Further studies are required to define the molecular mechanism by which VX-770 potentiates CFTR and to determine whether VX-770 also acts on other defective CFTR forms with other CFTR mutations.
3/16/2018 · Jih, K. Y. & Hwang, T. C. Vx-770 potentiates CFTR function by promoting decoupling between the gating cycle and ATP hydrolysis cycle.
9/1/2016 · These latest studies support the idea that VX-770 acts at the interface between CFTR’s TMDs and the lipid core of the membrane bilayer, and increases the Po by shifting the three vertical gating transitions depicted in Fig. 1A to favor the open states.
11/3/2017 · Two complementary types of drugs have been developed with different mechanisms of action to increase … in combination with ivacaftor ( VX-770 , … On the mechanism of gating defects caused …
View this document to explore the mechanism of action of ORKAMBI at the cellular level. Topics include: Normal CFTR protein function The F508del-CFTR mutation How lumacaftor and ivacaftor work together to increase the quantity, stability, and function of F508del-CFTR protein resulting in increased chloride ion transport MOA Downloadable Overview
