June 2016June 2016
New Product - Orkambi
Orkambi (lumacaftor, ivacaftor) contains lumacaftor which is a CFTR corrector that acts directly on F508del‑CFTR to improve its cellular processing and trafficking, thereby increasing the quantity of functional CFTR at the cell surface and ivacaftor which is a CFTR potentiator that facilitates increased chloride transport by potentiating the channel open probability (or gating) of the CFTR protein at the cell surface. The combined effect of lumacaftor and ivacaftor is increased quantity and function of F508del CFTR at the cell surface, resulting in increased chloride ion transport. The CFTR protein is a chloride channel present at the surface of epithelial cells in multiple organs. The F508del mutation impacts the CFTR protein in multiple ways, primarily by causing a defect in cellular processing and trafficking that reduces the quantity of CFTR at the cell surface. Orkambi is indicated for the treatment of cystic fibrosis in patients age 12 years and older who are homozygous for the F508del mutation in the CFTR gene. Orkambi is available as film coated tablets containing lumacaftor 200 mg/ ivacaftor 125 mg available as a box containing four cartons, each carton containing 7 blister strips of 4 tablets each (28’s) total of 112 tablets.
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