In vitro, we have recently shown the effectiveness of this strategy on Abcb11 missense variants: ivacaftor (VX-770, Kalydeco®), a clinically approved potentiating treatment for cystic fibrosis patients with some class III cystic fibrosis transmembrane conductance regulator (CFTR, ABCC7) variations, was able to potentiate the function of the Abcb11-T463I and Abcb11-A257V variants [10,11,12]. This evidence concerns the gene ABCB11 and cystic fibrosis.