Abstract: OBJECTIVE To explore the therapeutic effect of MMI-0100 on 3,5-diethoxycarboxyl-1,4-dihydro-2,4, 6-trimethylpyridine(DDC)-induced cholestatic liver injury in mice. METHODS Fifteen Balb/c mice were randomly divided into control group, model group(DDC) and treatment group(DDC+MMI-0100). There were 5 mice in each group. Mice in control group were fed with normal diet for 2 weeks, and mice in other two groups were fed with 0.1% DDC diet for 1 week and then given normal diet for another one week. After DDC diet for 1 week, mice in treatment group were intraperitoneally injected with 500 μg·kg^-1 of MMI-0100 every day for 1 week. Mice in control group and model group were given the same amount of sterile normal saline. The general conditions of liver of mice in each group were observed and recorded. The pathological changes of liver were observed by HE and Masson staining. Markers of biliary duct hyperplasia CK19 and Ki67 were detected by immunohistochemistry. The expression of α-SMA gene which related to liver fibrosis was detected by real-time quantitative PCR. RESULTS Compared with model group, liver fibrosis, inflammatory cell infiltration and the Knodell Score of mice all reduced significantly in the treatment group(P<0.01). In addition, the bile duct hyperplasia associated markers CK19 and Ki67 also decreased in the treatment group(P<0.05), and the mRNA expression level of α-SMA in liver was decreased(P<0.01). CONCLUSION MMI-0100 has a good therapeutic effect on DDC-induced mouse primary sclerosing cholangitis.