This is in line with previous data since cholestasis in cases of primary biliary cirrhosis or progressive familial intrahepatic cholestasis is associated with reduced OATP1B1 and OATP1B3 expression [12] and the concerted down-regulation of both bile salt uptake transporters is considered as a protective mechanism against hepatocellular injury caused by cytotoxic bile acids. This evidence concerns the gene SLCO1B3 and familial intrahepatic cholestasis.