In a model of sclerosing cholangitis and biliary fibrosis, genetic deletion of Cd39 exacerbates liver injury, fibrosis, and ductular reaction in multidrug-resistant-protein-2 (Mdr2)−/− mice and is associated with an increase in hepatic CD8 T cells; this phenotype can be reproduced upon administration of αβATP into CD39-sufficient Mdr2−/− mice, further supporting the immunomodulatory role of CD39 in cytotoxic CD8 cells (Peng et al., 2017). This evidence concerns the gene ENTPD1 and sclerosing cholangitis.