The key role of canalicular ABC transporters in bile secretion is highlighted by their implication in a wide range of diseases such as cholestasis (ABCB4, ABCB11), sitosterolemia (ABCG5/G8), Dubin–Johnson syndrome (ABCC2) and cancer (ABCB1) (Figure 1) [3]. This evidence concerns the gene ABCG2 and cholestasis.