In human neonates, ABCB11 deficiency can cause PFIC2, which leads to cholestasis and progressive cirrhosis in the first decade of life.9,10 In contrast to the human disease conditions, however, previous studies have shown that germline knockout of Abcb11 in mice does not lead to apparent features of liver injury because of the different hydrophobic nature of BA profiles in humans and rodents.26,30,31 We simultaneously knocked down the Cyp2c70 gene with Abcb11 to mimic the human BA profile. Here, ABCB11 is linked to cholestasis.