Answer
In Orkambi, lumacaftor corrects F508del-CFTR processing and trafficking, increasing functional protein at the cell surface, while ivacaftor potentiates CFTR channel opening to increase chloride transport; together they increase F508del-CFTR quantity and function, although their exact mechanisms are unknown.
Sources for this answer
- Orkambi 200 mg/125 mg film coated tabletsVertex Pharmaceuticals (Europe) Limited · emc · SmPC §5.1 Pharmacodynamic properties · Version 19Document updated
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