The combined effect of lumacaftor and ivacaftor is increased quantity and function of F508delCFTR at the cell surface, resulting in increased chloride ion transport. The exact mechanisms by which lumacaftor improves cellular processing and trafficking of F508delCFTR and ivacaftor potentiates F508del-CFTR are not known.
This medicinal substance has been classified in the anatomical therapeutic chemical (ATC) classification according to its main therapeutic use as follows:
| ATC code | Group title | Classification |
|---|---|---|
| R07AX30 | Ivacaftor and lumacaftor | R Respiratory system → R07 Other respiratory system products → R07A Other respiratory system products → R07AX Other respiratory system products |
Competent medicine agencies globally have authorized commercialization of this active ingredient according to these medication package inserts (MPIs):
| Title | Information Source | Document Type | |
|---|---|---|---|
| ORKAMBI Granules | European Medicines Agency (EU) | MPI, EU: SmPC | |
| ORKAMBI Film-coated tablet | European Medicines Agency (EU) | MPI, EU: SmPC |
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