Nonclinical end-point | Cell mutation | Ivacaftor | Lumacaftor | Orkambi |
Total ionic current (IT) (FRT cells) | G551D | ✓ | ||
F508del | ✓ | ✓ | ||
Total ionic current (IT) (HBE cells) | F508del | ✓ | ✓ | ✓ |
G551D/F508del | ✓ | |||
Open probability (PO) (NIH-3T3 cells) | G551D | ✓ | ||
F508del | ✓ | ✓ | ✓# | |
CFTR maturation (FRT cells) | F508del | ✓ | ||
CFTR maturation (HBE cells) | F508del | ✓ |
IT: total ionic current; FRT: Fischer rat thyroid; HBE: human bronchial epithelial; PO: open channel probability; CFTR: cystic fibrosis transmembrane conductance regulator. #: type of cells not reported.