In addition, the cathepsin Z was translocated from the bile canaliculus region of hepatocytes to the cytoplasm, particular in sepsis and Alagille syndrome (Fig. 7A) and immunofluorescence staining showed that cathepsin Z was no longer colocalized with LAMP1 in all three diseases (Fig. 7B). This evidence concerns the gene CTSZ and Alagille syndrome.