In this study, our interest in targeting NEDD8-activating enzyme for treatment of ICC was further stimulated by the observation that expression of neddylation pathway components, like NEDD8, NAE1, UBA3 and UBC12, was up-regulated in more than two third of human ICC and significantly positive correlations were found among them. The gene discussed is NAE1; the disease is intrahepatic cholangiocarcinoma.